http://publicationslist.org/data/ueli.suter/atom.xml Ueli Suterhttp://publicationslist.org/ueli.suter$basepathfavicon.icoRecent additions to Ueli Suter's PublicationsList.org pagehttp://publicationslist.org/publications.png2010-03-20T09:05:01Z http://publicationslist.org/ueli.suter/refid200 2010-03-20T09:04:35Z

Diameter, organization, and length of the myelin sheath are important determinants of the nerve conduction velocity, but the basic molecular mechanisms that control these parameters are only partially understood. Cell polarization is an essential feature of differentiated cells, and relies on a set of evolutionarily conserved cell polarity proteins. We investigated the molecular nature of myelin s...<br/><br/>Murat Ozçelik, Laurent Cotter, Claire Jacob, Jorge A Pereira, João B Relvas, Ueli Suter, Nicolas Tricaud (2010) <i>J Neurosci</i> <i></i> <i></i> 30: 11 4120-4131<br/>

http://publicationslist.org/ueli.suter/refid197 2010-03-06T07:42:43Z

Reorganization of the actin cytoskeleton is necessary for Schwann cell proliferation, migration and for the morphological changes associated with sorting, ensheathing and myelination of axons. Such reorganization requires regulated severing and depolymerization of actin filaments. Gelsolin is an actin filament severing protein expressed in many cell types including Schwann cells. Using Gelsolin kn...<br/><br/>Ana Filipa Gonçalves, Nuno G Dias, Martijn Moransard, Ricardo Correia, Jorge A Pereira, Walter Witke, Ueli Suter, João B Relvas (2010) <i>Glia</i> <i></i> <i></i> 58: 6 706-715<br/>

http://publicationslist.org/ueli.suter/refid199 2010-02-06T07:22:39Z

Patients with PMP22 deficiency present with focal sensory and motor deficits when peripheral nerves are stressed by mechanical force. It has been hypothesized that these focal deficits are due to mechanically induced conduction block (CB). To test this hypothesis, we induced 60-70% CB (defined by electrophysiological criteria) by nerve compression in an authentic mouse model of hereditary neuropat...<br/><br/>Yunhong Bai, Xuebao Zhang, Istvan Katona, Mario Andre Saporta, Michael E Shy, Heather A O'Malley, Lori L Isom, Ueli Suter, Jun Li (2010) <i>J Neurosci</i> 30: 2 600-608<br/>

http://publicationslist.org/ueli.suter/refid191 2009-05-30T07:22:15Z

During development, Schwann cells (SCs) interpret different extracellular cues to regulate their migration, proliferation, and the remarkable morphological changes associated with the sorting, ensheathment, and myelination of axons. Although interactions between extracellular matrix proteins and integrins are critical to some of these processes, the downstream signaling pathways they control are s...<br/><br/>Jorge A Pereira, Yves Benninger, Reto Baumann, Ana Filipa Gonçalves, Murat Ozçelik, Tina Thurnherr, Nicolas Tricaud, Dies Meijer, Reinhard Fässler, Ueli Suter, João B Relvas (2009) <i>J Cell Biol</i> <i></i> <i></i> 185: 1 147-161<br/>

http://publicationslist.org/ueli.suter/refid190 2009-07-18T06:19:28Z

Proteins controlling mitochondrial dynamics are often targeted to and anchored into the mitochondrial outer membrane (MOM) by their carboxyl-terminal tail-anchor domain (TA). However, it is not known whether the TA modulates protein function. GDAP1 is a mitochondrial fission factor with two neighboring hydrophobic domains each flanked by basic amino acids (aa). Here we define GDAP1 as TA MOM prote...<br/><br/>Konstanze M Wagner, Marcel Rüegg, Axel Niemann, Ueli Suter (2009) <i>PLoS One</i> 4: 4 <br/>

http://publicationslist.org/ueli.suter/refid188 2009-12-19T08:42:56Z

Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous disorder. All mendelian patterns of inheritance have been described. We identified a homozygous p.A335V mutation in the MED25 gene in an extended Costa Rican family with autosomal recessively inherited Charcot-Marie-Tooth neuropathy linked to the CMT2B2 locus in chromosome 19q13.3. MED25, also known as ARC92 and ACID1,...<br/><br/>Alejandro Leal, Kathrin Huehne, Finn Bauer, Heinrich Sticht, Philipp Berger, Ueli Suter, Bernal Morera, Gerardo Del Valle, James R Lupski, Arif Ekici, Francesca Pasutto, Sabine Endele, Ramiro Barrantes, Corinna Berghoff, Martin Berghoff, Bernhard Neundörfer, Dieter Heuss, Thomas Dorn, Peter Young, Lisa Santolin, Thomas Uhlmann, Michael Meisterernst, Michael Werner Sereda, Michael Sereda, Ruth Martha Stassart, Gerd Meyer Zu Horste, Klaus-Armin Nave, André Reis, Bernd Rautenstrauss (2009) <i>Neurogenetics</i> <i></i> <i></i> 10: 4 275-287<br/>

http://publicationslist.org/ueli.suter/refid192 2009-07-04T13:52:00Z

Auto-reactivity of T cells is largely prevented by central and peripheral tolerance. Nevertheless, immunization with certain self-antigens emulsified in CFA induces autoimmunity in rodents, suggesting that tolerance to some self-antigens is not robust. To investigate the fate of nervous system-specific CD8(+) T cells, which only recently came up as being important contributors for MS pathogenesis,...<br/><br/>Anita Schildknecht, Hans Christian Probst, Kathy D McCoy, Iris Miescher, Corinne Brenner, Dino P Leone, Ueli Suter, Pamela S Ohashi, Maries van den Broek (2009) <i>Eur J Immunol</i> 39: 6 1505-1515<br/>

http://publicationslist.org/ueli.suter/refid189 2009-05-16T07:22:13Z

The neural crest (NC) generates a variety of neural and non-neural tissues during vertebrate development. Both migratory NC cells and their target structures contain cells with stem cell features. Here we show that these populations of neural crest-derived stem cells (NCSCs) are differentially regulated by small Rho GTPases. Deletion of either Cdc42 or Rac1 in the NC results in size reduction of m...<br/><br/>Sebastian Fuchs, Dominik Herzog, Grzegorz Sumara, Stine Büchmann-Møller, Gianluca Civenni, Xunwei Wu, Anna Chrostek-Grashoff, Ueli Suter, Romeo Ricci, João B Relvas, Cord Brakebusch, Lukas Sommer (2009) <i>Cell Stem Cell</i> 4: 3 236-247<br/>

http://publicationslist.org/ueli.suter/refid194 2009-11-07T07:07:54Z

Charcot-Marie-Tooth disease type 4C (CMT4C) is an early-onset, autosomal recessive form of demyelinating neuropathy. The clinical manifestations include progressive scoliosis, delayed age of walking, muscular atrophy, distal weakness, and reduced nerve conduction velocity. The gene mutated in CMT4C disease, SH3TC2/KIAA1985, was recently identified; however, the function of the protein it encodes r...<br/><br/>Estelle Arnaud, Jennifer Zenker, Anne-Sophie de Preux Charles, Claudia Stendel, Andreas Roos, Jean-Jacques Médard, Nicolas Tricaud, Joachim Weis, Ueli Suter, Jan Senderek, Roman Chrast (2009) <i>Proc Natl Acad Sci U S A</i> 106: 41 17528-17533<br/>

http://publicationslist.org/ueli.suter/refid198 2010-03-06T07:41:48Z

Mutations in the GDAP1 gene lead to recessively or dominantly inherited peripheral neuropathies (Charcot-Marie-Tooth disease; CMT). Here, we demonstrate that GDAP1 is a mitochondrial fission factor whose activity is dependent on the fission factors Drp1 and Fis1. Unlike other mitochondrial fission factors, GDAP1 overexpression or knockdown does not influence the susceptibility of cells to apoptoti...<br/><br/>Axel Niemann, Konstanze Marion Wagner, Marcel Ruegg, Ueli Suter (2009) <i>Neurobiol Dis</i> <i></i> <i></i> 36: 3 509-520<br/>

http://publicationslist.org/ueli.suter/refid196 2010-01-09T12:46:42Z

Abstract Charcot-Marie-Tooth disease type 4B is caused by mutations in the genes encoding either the lipid phosphatase Myotubularin-Related-Protein-2 (MTMR2) or its regulatory binding partner MTMR13/SBF2. Mtmr2 dephosphorylates PI-3-P and PI-3,5-P2 to form phosphatidylinositol and PI-5-P, respectively, while Mtmr13/Sbf2 is an enzymatically inactive member of the myotubularin protein family. We hav...<br/><br/>Philipp Berger, Kristian Tersar, Kurt Ballmer-Hofer, Ueli Suter (2009) <i>J Cell Mol Med</i> <i></i> <i></i> : <br/>

http://publicationslist.org/ueli.suter/refid195 2009-11-21T07:21:56Z

Current opinion holds that pigment cells, melanocytes, are derived from neural crest cells produced at the dorsal neural tube and that migrate under the epidermis to populate all parts of the skin. Here, we identify growing nerves projecting throughout the body as a stem/progenitor niche containing Schwann cell precursors (SCPs) from which large numbers of skin melanocytes originate. SCPs arise as...<br/><br/>Igor Adameyko, Francois Lallemend, Jorge B Aquino, Jorge A Pereira, Piotr Topilko, Thomas Müller, Nicolas Fritz, Anna Beljajeva, Makoto Mochii, Isabel Liste, Dmitry Usoskin, Ueli Suter, Carmen Birchmeier, Patrik Ernfors (2009) <i>Cell</i> <i></i> <i></i> 139: 2 366-379<br/>

http://publicationslist.org/ueli.suter/refid3 2008-05-24T06:31:48Z

The question of how neurons and glial cells are generated during the development of the CNS has over time led to two alternative models: either neuroepithelial cells are capable of giving rise to neurons first and to glial cells at a later stage (switching model), or they are intrinsically committed to generate one or the other (segregating model). Using the developing diencephalon as a model and ...<br/><br/>Delphine Delaunay, Katharina Heydon, Ana Cumano, Markus H Schwab, Jean-Léon Thomas, Ueli Suter, Klaus-Armin Nave, Bernard Zalc, Nathalie Spassky (2008) <i>J Neurosci</i> <i></i> <i></i> 28: 10 2551-2562<br/>

http://publicationslist.org/ueli.suter/refid4 2008-05-24T06:31:48Z

Peripheral myelin formation depends on axonal signals that tightly control proliferation and differentiation of the associated Schwann cells. Here we demonstrate that the molecular program controlling proliferation of Schwann cells switches at birth. We have analyzed the requirements for three members of the cyclin-dependent kinase (cdk) family in Schwann cells using cdk-deficient mice. Mice lacki...<br/><br/>Suzana Atanasoski, Matthias Boentert, Lukas De Ventura, Hartmut Pohl, Constanze Baranek, Konstantin Beier, Peter Young, Mariano Barbacid, Ueli Suter (2008) <i>Mol Cell Neurosci</i> <i></i> <i></i> 37: 3 519-527<br/>

http://publicationslist.org/ueli.suter/refid2 2008-06-14T05:59:39Z

Prion diseases are untreatable neurodegenerative disorders characterized by accumulation of PrP(Sc), an aggregated isoform of the normal prion protein PrP(C). Here, we delivered the soluble prion antagonist PrP-Fc(2) to the brains of mice by lentiviral gene transfer. Although naïve mice developed scrapie at 175 +/- 5 days postintracerebral prion inoculation (dpi), gene transfer before inoculation...<br/><br/>Nicolas Genoud, David Ott, Nathalie Braun, Marco Prinz, Petra Schwarz, Ueli Suter, Didier Trono, Adriano Aguzzi (2008) <i>Am J Pathol</i> 172: 5 1287-1296<br/>

http://publicationslist.org/ueli.suter/refid185 2009-03-21T07:00:35Z

RhoGTPases are molecular switches that integrate extracellular signals to perform diverse cellular responses. This ability relies on the network of proteins regulating RhoGTPases activity and localization, and on the interaction of RhoGTPases with many different cellular effectors. Myelination is an ideal place for RhoGTPases regulation, as it is the result of fine orchestration of many stimuli fr...<br/><br/>M Laura Feltri, Ueli Suter, João B Relvas (2008) <i>Glia</i> <i></i> <i></i> 56: 14 1508-1517<br/>

http://publicationslist.org/ueli.suter/refid184 2008-09-06T05:33:57Z

Transforming growth factor beta (TGFbeta) promotes epithelial cell differentiation but induces Schwann cell proliferation. We show that the protooncogene Ski (Sloan-Kettering viral oncogene homologue) is an important regulator of these effects. TGFbeta down-regulates Ski in epithelial cells but not in Schwann cells. In Schwann cells but not in epithelial cells, retinoblastoma protein (Rb) is up-re...<br/><br/>Claire Jacob, Henrik Grabner, Suzana Atanasoski, Ueli Suter (2008) <i>J Cell Biol</i> <i></i> <i></i> 182: 3 519-530<br/>

http://publicationslist.org/ueli.suter/refid1 2009-01-17T07:18:54Z

Regulating the choice between neural stem cell maintenance versus differentiation determines growth and size of the developing brain. Here we identify TGF-beta signaling as a crucial factor controlling these processes. At early developmental stages, TGF-beta signal activity is localized close to the ventricular surface of the neuroepithelium. In the midbrain, but not in the forebrain, Tgfbr2 ablat...<br/><br/>Sven Falk, Heiko Wurdak, Lars M Ittner, Fabian Ille, Grzegorz Sumara, Marie-Theres Schmid, Kalina Draganova, Karl S Lang, Christian Paratore, Per Leveen, Ueli Suter, Stefan Karlsson, Walter Born, Romeo Ricci, Magdalena Götz, Lukas Sommer (2008) <i>Cell Stem Cell</i> <i></i> <i></i> 2: 5 472-483<br/>

http://publicationslist.org/ueli.suter/refid186 2009-02-07T06:48:54Z

A thorough knowledge of the cellular and molecular basis of the structure and function of peripheral nerves is of paramount importance not only for a better understanding of the fascinating biology of the peripheral nervous system but also for providing critical insights into the various diseases affecting peripheral nerves as the firm foundation of potential treatments. Genetic approaches in mode...<br/><br/>Sven Krause, Claudia Stendel, Jan Senderek, João B Relvas, Ueli Suter (2008) <i>J Peripher Nerv Syst</i> 13: 3 188-199<br/>

http://publicationslist.org/ueli.suter/refid9 2008-05-24T06:31:48Z

GTPases of the Rho subfamily are widely involved in the myelination of the vertebrate nervous system. Rho GTPase activity is temporally and spatially regulated by a set of specific guanine nucleotide exchange factors (GEFs). Here, we report that disruption of frabin/FGD4, a GEF for the Rho GTPase cell-division cycle 42 (Cdc42), causes peripheral nerve demyelination in patients with autosomal reces...<br/><br/>Claudia Stendel, Andreas Roos, Tine Deconinck, Jorge Pereira, Francois Castagner, Axel Niemann, Janbernd Kirschner, Rudolf Korinthenberg, Uwe-Peter Ketelsen, Esra Battaloglu, Yesim Parman, Garth Nicholson, Robert Ouvrier, Jürgen Seeger, Peter De Jonghe, Joachim Weis, Alexander Krüttgen, Sabine Rudnik-Schöneborn, Carsten Bergmann, Ueli Suter, Klaus Zerres, Vincent Timmerman, João B Relvas, Jan Senderek (2007) <i>Am J Hum Genet</i> <i></i> <i></i> 81: 1 158-164<br/>

http://publicationslist.org/ueli.suter/refid8 2008-05-24T06:31:48Z

During peripheral nervous system (PNS) myelination, Schwann cells must interpret extracellular cues to sense their environment and regulate their intrinsic developmental program accordingly. The pathways and mechanisms involved in this process are only partially understood. We use tissue-specific conditional gene targeting to show that members of the Rho GTPases, cdc42 and rac1, have different and...<br/><br/>Yves Benninger, Tina Thurnherr, Jorge A Pereira, Sven Krause, Xunwei Wu, Anna Chrostek-Grashoff, Dominik Herzog, Klaus-Armin Nave, Robin J M Franklin, Dies Meijer, Cord Brakebusch, Ueli Suter, João B Relvas (2007) <i>J Cell Biol</i> <i></i> <i></i> 177: 6 1051-1061<br/>

http://publicationslist.org/ueli.suter/refid11 2008-05-24T06:31:48Z

Multiple signaling pathways regulate proliferation and differentiation of neural progenitor cells during early development of the central nervous system (CNS). In the spinal cord, dorsal signaling by bone morphogenic protein (BMP) acts primarily as a patterning signal, while canonical Wnt signaling promotes cell cycle progression in stem and progenitor cells. However, overexpression of Wnt factors...<br/><br/>Fabian Ille, Suzana Atanasoski, Sven Falk, Lars M Ittner, David Märki, Stine Büchmann-Møller, Heiko Wurdak, Ueli Suter, Makoto M Taketo, Lukas Sommer (2007) <i>Dev Biol</i> <i></i> <i></i> 304: 1 394-408<br/>

http://publicationslist.org/ueli.suter/refid10 2008-05-24T06:31:48Z

Multiple molecular mechanisms influence nerve regeneration. Because serine proteases were shown to affect peripheral nerve regeneration, we performed nerve crush experiments to study synapse reinnervation in adult mice lacking the serpin protease nexin-1 (PN-1). PN-1 is a potent endogenous inhibitor of thrombin, trypsin, tissue plasminogen activators (tPAs), and urokinase plasminogen activators. C...<br/><br/>Maria Maddalena Lino, Suzana Atanasoski, Mirna Kvajo, Bérengère Fayard, Eliza Moreno, Hans Rudolf Brenner, Ueli Suter, Denis Monard (2007) <i>J Neurosci</i> <i></i> <i></i> 27: 14 3677-3685<br/>

http://publicationslist.org/ueli.suter/refid7 2008-05-24T06:31:48Z

Demyelination of the myelinated peripheral or central axon is a common pathophysiological step in the clinical manifestation of several human diseases of the peripheral and the central nervous system such as the majority of Charcot-Marie-Tooth syndromes and multiple sclerosis, respectively. The structural degradation of the axon insulating myelin sheath has profound consequences for ionic conducti...<br/><br/>Alejandro Heredia, Chin Chu Bui, Ueli Suter, Peter Young, Tilman E Schäffer (2007) <i>Neuroimage</i> <i></i> <i></i> 37: 4 1218-1226<br/>

http://publicationslist.org/ueli.suter/refid6 2008-05-24T06:31:48Z

Charcot-Marie-Tooth (CMT) disease denotes a large group of genetically heterogeneous hereditary motor and sensory neuropathies and ranks among the most common inherited neurological disorders. Mutations in the Myotubularin-Related Protein-2 (MTMR2) or MTMR13/Set-Binding Factor-2 (SBF2) genes are associated with the autosomal recessive disease subtypes CMT4B1 or CMT4B2. Both forms of CMT share simi...<br/><br/>Kristian Tersar, Matthias Boentert, Philipp Berger, Sonja Bonneick, Carsten Wessig, Klaus V Toyka, Peter Young, Ueli Suter (2007) <i>Hum Mol Genet</i> <i></i> <i></i> 16: 24 2991-3001<br/>

http://publicationslist.org/ueli.suter/refid5 2008-05-24T06:31:48Z

Recent research into the genetic basis and the molecular disease mechanisms of Charcot-Marie-Tooth disease (CMT), also called hereditary motor and sensory neuropathies, has highlighted phosphoinositides, membrane-tethered phosphorylated metabolites of phosphatidylinositol, as key regulatory molecules in peripheral nerves in health and disease. Enzymes that dephosphorylate the endosomal phosphoinos...<br/><br/>U Suter (2007) <i>Cell Mol Life Sci</i> <i></i> <i></i> 64: 24 3261-3265<br/>

http://publicationslist.org/ueli.suter/refid19 2008-05-24T06:31:48Z

Neuregulin/erbB signaling is critically required for survival and proliferation of Schwann cells as well as for establishing correct myelin thickness of peripheral nerves during development. In this study, we investigated whether erbB2 signaling in Schwann cells is also essential for the maintenance of myelinated peripheral nerves and for Schwann cell proliferation and survival after nerve injury....<br/><br/>Suzana Atanasoski, Steven S Scherer, Erich Sirkowski, Dino Leone, Alistair N Garratt, Carmen Birchmeier, Ueli Suter (2006) <i>J Neurosci</i> <i></i> <i></i> 26: 7 2124-2131<br/>

http://publicationslist.org/ueli.suter/refid21 2008-05-24T06:31:48Z

Mutations in myotubularin-related protein-2 (MTMR2) or MTMR13/set-binding factor-2 (SBF2) genes are responsible for the severe autosomal recessive hereditary neuropathies, Charcot-Marie-Tooth disease (CMT) types 4B1 and 4B2, both characterized by reduced nerve conduction velocities, focally folded myelin sheaths and demyelination. MTMRs form a large family of conserved dual-specific phosphatases w...<br/><br/>Philipp Berger, Imre Berger, Christiane Schaffitzel, Kristian Tersar, Benjamin Volkmer, Ueli Suter (2006) <i>Hum Mol Genet</i> <i></i> <i></i> 15: 4 569-579<br/>

http://publicationslist.org/ueli.suter/refid23 2008-05-24T06:31:48Z

Regulated cell proliferation is a crucial prerequisite for Schwann cells to achieve myelination in development and regeneration. In the present study, we have investigated the function of the cell cycle inhibitors p21 and p16 as potential regulators of Schwann cell proliferation, using p21- or p16-deficient mice. We report that both inhibitors are required for proper withdrawal of Schwann cells fr...<br/><br/>Suzana Atanasoski, Danielle Boller, Lukas De Ventura, Heidi Koegel, Matthias Boentert, Peter Young, Sabine Werner, Ueli Suter (2006) <i>Glia</i> <i></i> <i></i> 53: 2 147-157<br/>

http://publicationslist.org/ueli.suter/refid17 2008-05-24T06:31:48Z

We review the putative functions and malfunctions of proteins encoded by genes mutated in Charcot-Marie-Tooth disease (CMT; inherited motor and sensory neuropathies) in normal and affected peripheral nerves. Some proteins implicated in demyelinating CMT, peripheral myelin protein 22, protein zero (P0), and connexin32 (Cx32/GJB1) are crucial components of myelin. Periaxin is involved in connecting ...<br/><br/>Axel Niemann, Philipp Berger, Ueli Suter (2006) <i>Neuromolecular Med</i> <i></i> <i></i> 8: 1-2 217-242<br/>

http://publicationslist.org/ueli.suter/refid18 2008-05-24T06:31:48Z

Jagged1 is a ligand for members of the Notch family of receptors. Mutations in the human JAG1 gene are the major cause of Alagille syndrome, an autosomal dominant disorder affecting the liver, heart, eye, skeleton, kidneys, and craniofacial structures. Although expressed throughout mammalian embryonic development and in the adult, the function of Jagged1 in the central nervous system is not clear....<br/><br/>Mathias Weller, Nike Krautler, Ned Mantei, Ueli Suter, Verdon Taylor (2006) <i>Dev Neurosci</i> <i></i> <i></i> 28: 1-2 70-80<br/>

http://publicationslist.org/ueli.suter/refid12 2008-05-24T06:31:48Z

Given their accessibility, multipotent skin-derived cells might be useful for future cell replacement therapies. We describe the isolation of multipotent stem cell-like cells from the adult trunk skin of mice and humans that express the neural crest stem cell markers p75 and Sox10 and display extensive self-renewal capacity in sphere cultures. To determine the origin of these cells, we genetically...<br/><br/>Christine E Wong, Christian Paratore, María T Dours-Zimmermann, Ariane Rochat, Thomas Pietri, Ueli Suter, Dieter R Zimmermann, Sylvie Dufour, Jean Paul Thiery, Dies Meijer, Friedrich Beermann, Yann Barrandon, Lukas Sommer (2006) <i>J Cell Biol</i> <i></i> <i></i> 175: 6 1005-1015<br/>

http://publicationslist.org/ueli.suter/refid15 2008-05-24T06:31:48Z

Over the last 15 years, a number of mutations in a variety of genes have been identified that lead to inherited motor and sensory neuropathies (HMSN), also called Charcot-Marie-Tooth disease (CMT). In this review we will focus on the molecular and cellular mechanisms that cause the Schwann cell pathologies observed in dysmyelinating and demyelinating forms of CMT. In most instances, the underlying...<br/><br/>Philipp Berger, Axel Niemann, Ueli Suter (2006) <i>Glia</i> <i></i> <i></i> 54: 4 243-257<br/>

http://publicationslist.org/ueli.suter/refid14 2008-05-24T06:31:48Z

The formation of myelin sheaths in the CNS is the result of a complex series of events involving oligodendrocyte progenitor cell (OPC) proliferation, directed migration, and the morphological changes associated with axon ensheathment and myelination. To examine the role of Rho GTPases in oligodendrocyte biology, we have used a conditional tissue-specific gene-targeting approach. Ablation of Cdc42 ...<br/><br/>Tina Thurnherr, Yves Benninger, Xunwei Wu, Anna Chrostek, Sven M Krause, Klaus-Armin Nave, Robin J M Franklin, Cord Brakebusch, Ueli Suter, João B Relvas (2006) <i>J Neurosci</i> <i></i> <i></i> 26: 40 10110-10119<br/>

http://publicationslist.org/ueli.suter/refid16 2008-05-24T06:31:48Z

Previous reports, including transplantation experiments using dominant-negative inhibition of beta1-integrin signaling in oligodendrocyte progenitor cells, suggested that beta1-integrin signaling is required for myelination. Here, we test this hypothesis using conditional ablation of the beta1-integrin gene in oligodendroglial cells during the development of the CNS. This approach allowed us to st...<br/><br/>Yves Benninger, Holly Colognato, Tina Thurnherr, Robin J M Franklin, Dino P Leone, Suzana Atanasoski, Klaus-Armin Nave, Charles Ffrench-Constant, Ueli Suter, João B Relvas (2006) <i>J Neurosci</i> <i></i> <i></i> 26: 29 7665-7673<br/>

http://publicationslist.org/ueli.suter/refid28 2008-05-24T06:31:48Z

Canonical Wnt signaling instructively promotes sensory neurogenesis in early neural crest stem cells (eNCSCs) (Lee, H.Y., M. Kleber, L. Hari, V. Brault, U. Suter, M.M. Taketo, R. Kemler, and L. Sommer. 2004. Science. 303:1020-1023). However, during normal development Wnt signaling induces a sensory fate only in a subpopulation of eNCSCs while other cells maintain their stem cell features, despite ...<br/><br/>Maurice Kléber, Hye-Youn Lee, Heiko Wurdak, Johanna Buchstaller, Martin M Riccomagno, Lars M Ittner, Ueli Suter, Douglas J Epstein, Lukas Sommer (2005) <i>J Cell Biol</i> <i></i> <i></i> 169: 2 309-320<br/>

http://publicationslist.org/ueli.suter/refid29 2008-05-24T06:31:48Z

Point mutations affecting PMP22 can cause hereditary demyelinating and dysmyelinating peripheral neuropathies. In addition, duplication and deletion of PMP22 are associated with Charcot-Marie-Tooth disease Type 1A (CMT1A) and Hereditary Neuropathy with Liability to Pressure Palsy (HNPP), respectively. This study was designed to elucidate disease processes caused by misexpression of Pmp22 and, at t...<br/><br/>Guya Giambonini-Brugnoli, Johanna Buchstaller, Lukas Sommer, Ueli Suter, Ned Mantei (2005) <i>Neurobiol Dis</i> <i></i> <i></i> 18: 3 656-668<br/>

http://publicationslist.org/ueli.suter/refid30 2008-05-24T06:31:48Z

Specific inactivation of TGFbeta signaling in neural crest stem cells (NCSCs) results in cardiovascular defects and thymic, parathyroid, and craniofacial anomalies. All these malformations characterize DiGeorge syndrome, the most common microdeletion syndrome in humans. Consistent with a role of TGFbeta in promoting non-neural lineages in NCSCs, mutant neural crest cells migrate into the pharyngea...<br/><br/>Heiko Wurdak, Lars M Ittner, Karl S Lang, Per Leveen, Ueli Suter, Jan A Fischer, Stefan Karlsson, Walter Born, Lukas Sommer (2005) <i>Genes Dev</i> <i></i> <i></i> 19: 5 530-535<br/>

http://publicationslist.org/ueli.suter/refid31 2008-05-24T06:31:48Z

Jagged1-mediated Notch signaling has been suggested to be critically involved in hematopoietic stem cell (HSC) self-renewal. Unexpectedly, we report here that inducible Cre-loxP-mediated inactivation of the Jagged1 gene in bone marrow progenitors and/or bone marrow (BM) stromal cells does not impair HSC self-renewal or differentiation in all blood lineages. Mice with simultaneous inactivation of J...<br/><br/>Stéphane J C Mancini, Ned Mantei, Alexis Dumortier, Ueli Suter, H Robson MacDonald, Freddy Radtke (2005) <i>Blood</i> <i></i> <i></i> 105: 6 2340-2342<br/>

http://publicationslist.org/ueli.suter/refid27 2008-05-24T06:31:48Z

Neural stem cells give rise to undifferentiated nestin-positive progenitors that undergo extensive cell division before differentiating into neuronal and glial cells. The precise control of this process is likely to be, at least in part, controlled by instructive cues originating from the extracellular environment. Some of these cues are interpreted by the integrin family of extracellular matrix r...<br/><br/>Dino P Leone, João B Relvas, Lia S Campos, Silvio Hemmi, Cord Brakebusch, Reinhard Fässler, Charles Ffrench-Constant, Ueli Suter (2005) <i>J Cell Sci</i> <i></i> <i></i> 118: Pt 12 2589-2599<br/>

http://publicationslist.org/ueli.suter/refid22 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease (CMT) comprises a family of clinically and genetically very heterogeneous hereditary peripheral neuropathies and is one of the most common inherited neurological disorders. We have generated a mouse model for CMT type 4B1 using embryonic stem cell technology. To this end, we introduced a stop codon into the Mtmr2 locus within exon 9, at the position encoding amino acid ...<br/><br/>Sonja Bonneick, Matthias Boentert, Philipp Berger, Suzana Atanasoski, Ned Mantei, Carsten Wessig, Klaus V Toyka, Peter Young, Ueli Suter (2005) <i>Hum Mol Genet</i> <i></i> <i></i> 14: 23 3685-3695<br/>

http://publicationslist.org/ueli.suter/refid26 2008-05-24T06:31:48Z

The contribution of Fas (CD95/APO-1) to cell death mechanisms of differentiated neurons is controversially discussed. Rat cerebellar granule neurons (CGNs) express high levels of Fas in vitro but are resistant to FasL (CD95L/APO-1L/CD178)-induced apoptosis. We here show that this resistance was mediated by a phosphatidylinositol 3-kinase (PI 3-kinase)-Akt/protein kinase B (PKB)-dependent expressio...<br/><br/>Christoph P Beier, Jörg Wischhusen, Marc Gleichmann, Ellen Gerhardt, Ana Pekanovic, Andreas Krueger, Verdon Taylor, Ueli Suter, Peter H Krammer, Matthias Endres, Michael Weller, Jörg B Schulz (2005) <i>J Neurosci</i> <i></i> <i></i> 25: 29 6765-6774<br/>

http://publicationslist.org/ueli.suter/refid24 2008-05-24T06:31:48Z

Mutations in GDAP1 lead to severe forms of the peripheral motor and sensory neuropathy, Charcot-Marie-Tooth disease (CMT), which is characterized by heterogeneous phenotypes, including pronounced axonal damage and demyelination. We show that neurons and Schwann cells express ganglioside-induced differentiation associated protein 1 (GDAP1), which suggest that both cell types may contribute to the m...<br/><br/>Axel Niemann, Marcel Ruegg, Veronica La Padula, Angelo Schenone, Ueli Suter (2005) <i>J Cell Biol</i> <i></i> <i></i> 170: 7 1067-1078<br/>

http://publicationslist.org/ueli.suter/refid20 2008-05-24T06:31:48Z

BACKGROUND: Development of the eye depends partly on the periocular mesenchyme derived from the neural crest (NC), but the fate of NC cells in mammalian eye development and the signals coordinating the formation of ocular structures are poorly understood. RESULTS: Here we reveal distinct NC contributions to both anterior and posterior mesenchymal eye structures and show that TGFbeta signaling in t...<br/><br/>Lars M Ittner, Heiko Wurdak, Kerstin Schwerdtfeger, Thomas Kunz, Fabian Ille, Per Leveen, Tord A Hjalt, Ueli Suter, Stefan Karlsson, Farhad Hafezi, Walter Born, Lukas Sommer (2005) <i>J Biol</i> <i></i> <i></i> 4: 3 <br/>

http://publicationslist.org/ueli.suter/refid25 2008-05-24T06:31:48Z

Neural stem cells (NSCs) in the postnatal mammalian brain self-renew and are a source of neurons and glia. To date, little is known about the molecular and cellular mechanisms regulating the maintenance and differentiation of these multipotent progenitors. We show that Jagged1 is required by mitotic cells in the subventricular zone (SVZ) and stimulates self-renewal of multipotent epidermal growth ...<br/><br/>Yves Nyfeler, Robert D Kirch, Ned Mantei, Dino P Leone, Freddy Radtke, Ueli Suter, Verdon Taylor (2005) <i>EMBO J</i> <i></i> <i></i> 24: 19 3504-3515<br/>

http://publicationslist.org/ueli.suter/refid40 2008-05-24T06:31:48Z

Schwann cells develop from multipotent neural crest stem cells and are important for neuronal survival, maintenance of axonal integrity, and myelination. We used transgenic mice expressing green fluorescent protein in a tissue-specific manner to isolate viable, pure populations of neural crest stem cells and developing Schwann cells, which are not readily accessible by microdissection. Starting wi...<br/><br/>Johanna Buchstaller, Lukas Sommer, Matthias Bodmer, Reinhard Hoffmann, Ueli Suter, Ned Mantei (2004) <i>J Neurosci</i> <i></i> <i></i> 24: 10 2357-2365<br/>

http://publicationslist.org/ueli.suter/refid41 2008-05-24T06:31:48Z

Notch is a key regulator of vertebrate neurogenesis and the cytoplasmic adaptor protein Numb is a modulator of the Notch signaling pathway. To address the role of murine Numb in development of the central nervous system, we used a conditional gene ablation approach. We show that Numb is involved in the maturation of cerebellar granule cells. Although the specification of neural cell fates in the c...<br/><br/>Anne-Laurence Klein, Olav Zilian, Ueli Suter, Verdon Taylor (2004) <i>Dev Biol</i> <i></i> <i></i> 266: 1 161-177<br/>

http://publicationslist.org/ueli.suter/refid42 2008-05-24T06:31:48Z

Wnt signaling has recently emerged as a key factor in controlling stem cell expansion. In contrast, we show here that Wnt/beta-catenin signal activation in emigrating neural crest stem cells (NCSCs) has little effect on the population size and instead regulates fate decisions. Sustained beta-catenin activity in neural crest cells promotes the formation of sensory neural cells in vivo at the expens...<br/><br/>Hye-Youn Lee, Maurice Kléber, Lisette Hari, Véronique Brault, Ueli Suter, Makoto M Taketo, Rolf Kemler, Lukas Sommer (2004) <i>Science</i> <i></i> <i></i> 303: 5660 1020-1023<br/>

http://publicationslist.org/ueli.suter/refid38 2008-05-24T06:31:48Z

E Bertini, V Biancalana, A Bolino, A Buj Bello, M Clague, P Guicheney, H Jungbluth, W Kress, A Musaro', H Nandurkar, L Pirola, N Romero, J Senderek, U Suter, C Sewry, H Tronchere, C Wallgren-Pettersson, M J Wishart, J Laporte (2004) <i>Neuromuscul Disord</i> <i></i> <i></i> 14: 6 387-396<br/>

http://publicationslist.org/ueli.suter/refid37 2008-05-24T06:31:48Z

The emerging evidence that stem cells develop in specialised niches highlights the potential role of environmental factors in their regulation. Here we examine the role of beta1 integrin/extracellular matrix interactions in neural stem cells. We find high levels of beta1 integrin expression in the stem-cell containing regions of the embryonic CNS, with associated expression of the laminin alpha2 c...<br/><br/>Lia S Campos, Dino P Leone, Joao B Relvas, Cord Brakebusch, Reinhard Fässler, Ueli Suter, Charles ffrench-Constant (2004) <i>Development</i> <i></i> <i></i> 131: 14 3433-3444<br/>

http://publicationslist.org/ueli.suter/refid32 2008-05-24T06:31:48Z

Mutations in the gene encoding N-myc downstream-regulated gene-1 (NDRG1) lead to truncations of the encoded protein and are associated with an autosomal recessive demyelinating neuropathy--hereditary motor and sensory neuropathy-Lom. NDRG1 protein is highly expressed in peripheral nerve and is localized in the cytoplasm of myelinating Schwann cells, including the paranodes and Schmidt-Lanterman in...<br/><br/>Philipp Berger, Erich E Sirkowski, Steven S Scherer, Ueli Suter (2004) <i>Neurobiol Dis</i> <i></i> <i></i> 17: 2 290-299<br/>

http://publicationslist.org/ueli.suter/refid34 2008-05-24T06:31:48Z

Schwann cell proliferation and subsequent differentiation to nonmyelinating and myelinating cells are closely linked processes. Elucidating the molecular mechanisms that control these events is key to the understanding of nerve development, regeneration, nerve-sheath tumors, and neuropathies. We define the protooncogene Ski, an inhibitor of TGF-beta signaling, as an essential component of the mach...<br/><br/>Suzana Atanasoski, Lucia Notterpek, Hye-Youn Lee, François Castagner, Peter Young, Markus U Ehrengruber, Dies Meijer, Lukas Sommer, Ed Stavnezer, Clemencia Colmenares, Ueli Suter (2004) <i>Neuron</i> <i></i> <i></i> 43: 4 499-511<br/>

http://publicationslist.org/ueli.suter/refid33 2008-05-24T06:31:48Z

The myelin and lymphocyte protein (MAL) is a tetraspan raft-associated proteolipid predominantly expressed by oligodendrocytes and Schwann cells. We show that genetic ablation of mal resulted in cytoplasmic inclusions within compact myelin, paranodal loops that are everted away from the axon, and disorganized transverse bands at the paranode--axon interface in the adult central nervous system. The...<br/><br/>Nicole Schaeren-Wiemers, Annick Bonnet, Michael Erb, Beat Erne, Udo Bartsch, Frances Kern, Ned Mantei, Diane Sherman, Ueli Suter (2004) <i>J Cell Biol</i> <i></i> <i></i> 166: 5 731-742<br/>

http://publicationslist.org/ueli.suter/refid35 2008-05-24T06:31:48Z

The reasons for the eventual failure of repair mechanisms in multiple sclerosis are unknown. The presence of precursor and immature oligodendrocytes in some non-repairing lesions suggests a mechanism in which these cells either receive insufficient differentiation signals or are exposed to differentiation inhibitors. Jagged signalling via Notch receptors on oligodendrocyte precursor cells (OPCs) i...<br/><br/>Mark F Stidworthy, Stephane Genoud, Wen-Wu Li, Dino P Leone, Ned Mantei, Ueli Suter, Robin J M Franklin (2004) <i>Brain</i> <i></i> <i></i> 127: Pt 9 1928-1941<br/>

http://publicationslist.org/ueli.suter/refid48 2008-05-24T06:31:48Z

Mutations in the myotubularin (MTM)-related protein 2 (MTMR2) gene are responsible for the severe autosomal recessive neuropathy Charcot-Marie-Tooth disease type 4B1. MTMR2 belongs to the MTM family of dual-specific phosphatases that use phosphatidylinositol (PI) 3,5-bisphosphate [PI(3,5)P2] and PI 3-phosphate [PI(3)P] as their substrate. Because these substrates are localized in the membrane bila...<br/><br/>Philipp Berger, Christiane Schaffitzel, Imre Berger, Nenad Ban, Ueli Suter (2003) <i>Proc Natl Acad Sci U S A</i> <i></i> <i></i> 100: 21 12177-12182<br/>

http://publicationslist.org/ueli.suter/refid50 2008-05-24T06:31:48Z

The demyelinating toxin cuprizone is used increasingly in mouse studies of central nervous system remyelination. The value of this model for such studies depends on an accurate description of its quantifiable features. We therefore investigated histology and ultrastructure during the early oligodendrocyte differentiation phase of remyelination in mice given cuprizone and allowed to recover for 2 w...<br/><br/>Mark F Stidworthy, Stephane Genoud, Ueli Suter, Ned Mantei, Robin J M Franklin (2003) <i>Brain Pathol</i> <i></i> <i></i> 13: 3 329-339<br/>

http://publicationslist.org/ueli.suter/refid52 2008-05-24T06:31:48Z

Inducible transgenesis provides a valuable technique for the analysis of gene function in vivo. We report the generation and characterization of mouse lines carrying glia lineage-specific transgenes expressing an improved variant of the tamoxifen-inducible Cre recombinase, CreERT2, where the recombinase is fused to a mutated ligand binding domain of the human estrogen receptor. Using a PLP-CreERT2...<br/><br/>Dino P Leone, Stéphane Genoud, Suzana Atanasoski, Reinhard Grausenburger, Philipp Berger, Daniel Metzger, Wendy B Macklin, Pierre Chambon, Ueli Suter (2003) <i>Mol Cell Neurosci</i> <i></i> <i></i> 22: 4 430-440<br/>

http://publicationslist.org/ueli.suter/refid47 2008-05-24T06:31:48Z

E-cadherin is thought to mediate intercellular adhesion in the mammalian epidermis and in hair follicles as the adhesive component of adherens junctions. We have tested this role of E-cadherin directly by conditional gene ablation in the mouse. We show that postnatal loss of E-cadherin in keratinocytes leads to a loss of adherens junctions and altered epidermal differentiation without accompanying...<br/><br/>Peter Young, Oreda Boussadia, Hartmut Halfter, Richard Grose, Philipp Berger, Dino P Leone, Horst Robenek, Patrick Charnay, Rolf Kemler, Ueli Suter (2003) <i>EMBO J</i> <i></i> <i></i> 22: 21 5723-5733<br/>

http://publicationslist.org/ueli.suter/refid49 2008-05-24T06:31:48Z

Ueli Suter, Steven S Scherer (2003) <i>Nat Rev Neurosci</i> <i></i> <i></i> 4: 9 714-726<br/>

http://publicationslist.org/ueli.suter/refid46 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy. The predominant subtype, CMT-1A, accounts for more than 50% of all cases and is associated with an interstitial chromosomal duplication of 17p12 (refs. 2,3). We have generated a model of CMT-1A by introducing extra copies of the responsible disease gene, Pmp22 (encoding the peripheral myelin protein of 22 kDa), into transge...<br/><br/>Michael W Sereda, Gerd Meyer zu Hörste, Ueli Suter, Naureen Uzma, Klaus-Armin Nave (2003) <i>Nat Med</i> <i></i> <i></i> 9: 12 1533-1537<br/>

http://publicationslist.org/ueli.suter/refid43 2008-05-24T06:31:48Z

Charcot-Marie-Tooth (CMT) disease serves as the summary term for the most frequent forms of inherited peripheral neuropathies that affect motor and sensory nerves. In the last 12 years, 14 genes have been identified that cause different CMT subforms. The genes found initially are predominantly responsible for demyelinating and dysmyelinating neuropathies. Genes affected in axonal and rare forms of...<br/><br/>P Young, U Suter (2003) <i>Cell Mol Life Sci</i> <i></i> <i></i> 60: 12 2547-2560<br/>

http://publicationslist.org/ueli.suter/refid44 2008-05-24T06:31:48Z

Genetic disease mechanisms in the demyelinating peripheral neuropathies Charcot-Marie-Tooth disease type 1A (CMTA) and hereditary neuropathy with liability to pressure palsies (HNPP) as well as transgenic animals with altered PMP22 gene dosage revealed that alterations in PMP22 gene expression have profound effects on the development and maintenance of peripheral nerves. Consequently, the regulati...<br/><br/>Marcel Maier, François Castagner, Philipp Berger, Ueli Suter (2003) <i>Mol Cell Neurosci</i> <i></i> <i></i> 24: 3 803-817<br/>

http://publicationslist.org/ueli.suter/refid45 2008-05-24T06:31:48Z

Without medical progress, dementing diseases such as Alzheimer's disease will become one of the main causes of disability. Preventing or delaying them has thus become a real challenge for biomedical research. Steroids offer interesting therapeutical opportunities for promoting successful aging because of their pleiotropic effects in the nervous system: they regulate main neurotransmitter systems, ...<br/><br/>M Schumacher, S Weill-Engerer, P Liere, F Robert, R J M Franklin, L M Garcia-Segura, J J Lambert, W Mayo, R C Melcangi, A Parducz, U Suter, C Carelli, E E Baulieu, Y Akwa (2003) <i>Prog Neurobiol</i> <i></i> <i></i> 71: 1 3-29<br/>

http://publicationslist.org/ueli.suter/refid65 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease (CMT), also named hereditary motor and sensory neuropathies, includes a clinically and genetically heterogeneous group of disorders affecting the peripheral nervous system. Traditionally, the different classes of CMT have been divided into demyelinating forms (CMT1, CMT3, and CMT4) and axonal forms (CMT2), a clinically very useful distinction. However, investigations of...<br/><br/>Philipp Berger, Peter Young, Ueli Suter (2002) <i>Neurogenetics</i> <i></i> <i></i> 4: 1 1-15<br/>

http://publicationslist.org/ueli.suter/refid63 2008-05-24T06:31:48Z

Many steps of peripheral glia development appear to be regulated by neuregulin1 (NRG1) signaling but the exact roles of the different NRG1 isoforms in these processes remain to be determined. While glial growth factor 2 (GGF2), a NRG1 type II isoform, is able to induce a satellite glial fate in neural crest stem cells, targeted mutations in mice have revealed a prominent role of NRG1 type III isof...<br/><br/>Rainer Leimeroth, Christian Lobsiger, Agnes Lüssi, Verdon Taylor, Ueli Suter, Lukas Sommer (2002) <i>Dev Biol</i> <i></i> <i></i> 246: 2 245-258<br/>

http://publicationslist.org/ueli.suter/refid62 2008-05-24T06:31:48Z

Minor changes in PMP22 gene dosage have profound effects on the development and maintenance of peripheral nerves. This is evident from the genetic disease mechanisms in Charcot-Marie-Tooth disease type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) as well as transgenic animals with altered PMP22 gene dosage. Thus, regulation of PMP22 is a crucial aspect in understa...<br/><br/>Marcel Maier, Philipp Berger, Klaus-Armin Nave, Ueli Suter (2002) <i>Mol Cell Neurosci</i> <i></i> <i></i> 20: 1 93-109<br/>

http://publicationslist.org/ueli.suter/refid66 2008-05-24T06:31:48Z

Schwann cells are the major glial population of the vertebrate peripheral nervous system. In the adult, they build a protecting sheath around neuronal processes and myelinate large-caliber axons. Already early in development, Schwann cells and neurons establish close contacts. Later development and the maintenance of peripheral nerves are crucially dependent on the controlled bi-directional dialog...<br/><br/>Christian S Lobsiger, Verdon Taylor, Ueli Suter (2002) <i>Biol Chem</i> <i></i> <i></i> 383: 2 245-253<br/>

http://publicationslist.org/ueli.suter/refid69 2008-05-24T06:31:48Z

The mechanisms that guide progenitor cell fate and differentiation in the vertebrate central nervous system (CNS) are poorly understood. Gain-of-function experiments suggest that Notch signaling is involved in the early stages of mammalian neurogenesis. On the basis of the expression of Notch1 by putative progenitor cells of the vertebrate CNS, we have addressed directly the role of Notch1 in the ...<br/><br/>Simone Lütolf, Freddy Radtke, Michel Aguet, Ueli Suter, Verdon Taylor (2002) <i>Development</i> <i></i> <i></i> 129: 2 373-385<br/>

http://publicationslist.org/ueli.suter/refid61 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease (CMT) comprises a heterogeneous group of disorders. The most frequent subtype is caused by increased PMP22 gene dosage or missense point mutations affecting the PMP22 gene (CMT type 1A; CMT1A). Animal models in rat and mouse with the corresponding PMP22 alterations are available and mimic many aspects of the human diseases. Detailed examinations of the animal mutants, t...<br/><br/>Marcel Maier, Philipp Berger, Ueli Suter (2002) <i>J Anat</i> <i></i> <i></i> 200: 4 357-366<br/>

http://publicationslist.org/ueli.suter/refid68 2008-05-24T06:31:48Z

Overexpression of PMP22 is responsible for the most common form of inherited neuropathy, Charcot-Marie-Tooth disease (CMT) type 1A. The PMP22-transgenic rat (CMT rat) is an animal model of CMT1A, and its peripheral nerves show the characteristic features of ongoing demyelination and remyelination that is also seen in CMT1A patients. Since Schwann cell proliferation is a prominent feature of periph...<br/><br/>Suzana Atanasoski, Steven S Scherer, Klaus-Armin Nave, Ueli Suter (2002) <i>J Neurosci Res</i> <i></i> <i></i> 67: 4 443-449<br/>

http://publicationslist.org/ueli.suter/refid67 2008-05-24T06:31:48Z

Multipotent stem cells must generate various differentiated cell types in correct number and sequence during neural development. In the peripheral nervous system (PNS), this involves the formation of postmigratory progenitor cell types which maintain multipotency and are able to give rise to neural and non-neural cells in response to instructive growth factors. We propose that fate restrictions in...<br/><br/>Christian Paratore, Lilian Hagedorn, Julien Floris, Lisette Hari, Maurice Kléber, Ueli Suter, Lukas Sommer (2002) <i>Int J Dev Biol</i> <i></i> <i></i> 46: 1 193-200<br/>

http://publicationslist.org/ueli.suter/refid64 2008-05-24T06:31:48Z

Mutations in the gene encoding myotubularin-related protein 2 (MTMR2) are responsible for autosomal recessive Charcot-Marie-Tooth disease type 4B1 (CMT4B1), a severe hereditary motor and sensory neuropathy characterized by focally folded myelin sheaths and demyelination. MTMR2 belongs to the myotubularin family, which is characterized by the presence of a phosphatase domain. Myotubularin (MTM), th...<br/><br/>Philipp Berger, Sonja Bonneick, Susan Willi, Matthias Wymann, Ueli Suter (2002) <i>Hum Mol Genet</i> <i></i> <i></i> 11: 13 1569-1579<br/>

http://publicationslist.org/ueli.suter/refid54 2008-05-24T06:31:48Z

Hirschsprung disease, or congenital megacolon, is characterized by aganglionosis of the terminal bowel, which leads to intestinal obstruction and chronic constipation. Several genes involved in the disease have been identified. In particular, haploinsufficiency of SOX10, which encodes a transcription factor, results in megacolon, often in combination with other disorders. Although Hirschsprung dis...<br/><br/>Christian Paratore, Christof Eichenberger, Ueli Suter, Lukas Sommer (2002) <i>Hum Mol Genet</i> <i></i> <i></i> 11: 24 3075-3085<br/>

http://publicationslist.org/ueli.suter/refid53 2008-05-24T06:31:48Z

Beta-catenin plays a pivotal role in cadherin-mediated cell adhesion. Moreover, it is a downstream signaling component of Wnt that controls multiple developmental processes such as cell proliferation, apoptosis, and fate decisions. To study the role of beta-catenin in neural crest development, we used the Cre/loxP system to ablate beta-catenin specifically in neural crest stem cells. Although seve...<br/><br/>Lisette Hari, Véronique Brault, Maurice Kléber, Hye-Youn Lee, Fabian Ille, Rainer Leimeroth, Christian Paratore, Ueli Suter, Rolf Kemler, Lukas Sommer (2002) <i>J Cell Biol</i> <i></i> <i></i> 159: 5 867-880<br/>

http://publicationslist.org/ueli.suter/refid60 2008-05-24T06:31:48Z

Notch signaling plays a pivotal role in the regulation of vertebrate neurogenesis. However, in vitro experiments suggest that Notch1 may also be involved in the regulation of later stages of brain development. We have addressed putative roles in the central nervous system by examining the expression of Notch signaling cascade components in the postnatal mouse brain. In situ mRNA hybridization reve...<br/><br/>Gila Stump, André Durrer, Anne-Laurence Klein, Simone Lütolf, Ueli Suter, Verdon Taylor (2002) <i>Mech Dev</i> <i></i> <i></i> 114: 1-2 153-159<br/>

http://publicationslist.org/ueli.suter/refid55 2008-05-24T06:31:48Z

We have previously identified and characterized a cDNA coding for neural membrane protein 35 (NMP35). We showed that NMP35 mRNA is predominantly expressed in the adult CNS with a neuronal expression pattern. Functional analysis indicates that the human homologue of NMP35, Lifeguard, plays a role in Fas-mediated cell death. In this study we used affinity-purified antibodies raised against the putat...<br/><br/>Beat Schweitzer, Ueli Suter, Verdon Taylor (2002) <i>Brain Res Mol Brain Res</i> <i></i> <i></i> 107: 1 47-56<br/>

http://publicationslist.org/ueli.suter/refid56 2008-05-24T06:31:48Z

The calcium-dependent adhesion protein E-cadherin is present in noncompacted regions of myelin sheaths in the peripheral nervous system. There, it is localized to electron-dense structures between membranes of the same Schwann cell referred to as autotypic adherens junctions. It has been suggested that the failure of E-cadherin-mediated adhesion might cause demyelination that proceeds in certain p...<br/><br/>Peter Young, Oreda Boussadia, Philipp Berger, Dino P Leone, Patrick Charnay, Rolf Kemler, Ueli Suter (2002) <i>Mol Cell Neurosci</i> <i></i> <i></i> 21: 2 341-351<br/>

http://publicationslist.org/ueli.suter/refid59 2008-05-24T06:31:48Z

We have selectively inhibited Notch1 signaling in oligodendrocyte precursors (OPCs) using the Cre/loxP system in transgenic mice to investigate the role of Notch1 in oligodendrocyte (OL) development and differentiation. Early development of OPCs appeared normal in the spinal cord. However, at embryonic day 17.5, premature OL differentiation was observed and ectopic immature OLs were present in the...<br/><br/>Stephane Genoud, Corinna Lappe-Siefke, Sandra Goebbels, Freddy Radtke, Michel Aguet, Steven S Scherer, Ueli Suter, Klaus-Armin Nave, Ned Mantei (2002) <i>J Cell Biol</i> <i></i> <i></i> 158: 4 709-718<br/>

http://publicationslist.org/ueli.suter/refid58 2008-05-24T06:31:48Z

The transcription factor Erm is a member of the Pea3 subfamily of Ets domain proteins that is expressed in multipotent neural crest cells, peripheral neurons, and satellite glia. A specific role of Erm during development has not yet been established. We addressed the function of Erm in neural crest development by forced expression of a dominant-negative form of Erm. Functional inhibition of Erm in...<br/><br/>Christian Paratore, Guya Brugnoli, Hye-Youn Lee, Ueli Suter, Lukas Sommer (2002) <i>Dev Biol</i> <i></i> <i></i> 250: 1 168-180<br/>

http://publicationslist.org/ueli.suter/refid57 2008-05-24T06:31:48Z

Injured axons in mammalian peripheral nerves often regenerate successfully over long distances, in contrast to axons in the brain and spinal cord (CNS). Neurite growth-inhibitory proteins, including the recently cloned membrane protein Nogo-A, are enriched in the CNS, in particular in myelin. Nogo-A is not detectable in peripheral nerve myelin. Using regulated transgenic expression of Nogo-A in pe...<br/><br/>Caroline Pot, Marjo Simonen, Oliver Weinmann, Lisa Schnell, Franziska Christ, Sascha Stoeckle, Philipp Berger, Thomas Rülicke, Ueli Suter, Martin E Schwab (2002) <i>J Cell Biol</i> <i></i> <i></i> 159: 1 29-35<br/>

http://publicationslist.org/ueli.suter/refid77 2008-05-24T06:31:48Z

BACKGROUND: Notch signaling regulates multiple differentiation processes and cell fate decisions during both invertebrate and vertebrate development. Numb encodes an intracellular protein that was shown in Drosophila to antagonize Notch signaling at binary cell fate decisions of certain cell lineages. Although overexpression experiments suggested that Numb might also antagonize some Notch activity...<br/><br/>O Zilian, C Saner, L Hagedorn, H Y Lee, E Säuberli, U Suter, L Sommer, M Aguet (2001) <i>Curr Biol</i> <i></i> <i></i> 11: 7 494-501<br/>

http://publicationslist.org/ueli.suter/refid78 2008-05-24T06:31:48Z

During myelin formation, membrane-associated proteins have to be sorted and transported in specified membrane regions such as compact and non-compact myelin membranes. One protein that may be involved in such a process is the Myelin and Lymphocyte protein MAL (VIP17/ MVP17). MAL was identified as a novel myelin membrane component expressed by oligodendrocytes and Schwann cells. Since MAL has been ...<br/><br/>J Caduff, S Sansano, A Bonnet, U Suter, N Schaeren-Wiemers (2001) <i>Microsc Res Tech</i> <i></i> <i></i> 52: 6 645-655<br/>

http://publicationslist.org/ueli.suter/refid75 2008-05-24T06:31:48Z

Dramatic progress has been made over recent years toward the elucidation of the mechanisms regulating lineage determination and cell survival in the developing peripheral nervous system. However, our understanding of Schwann cell development is limited. This is partly due to the difficulties in culturing primary Schwann cell precursor cells, the earliest developmental stage of the Schwann cell lin...<br/><br/>C S Lobsiger, P M Smith, J Buchstaller, B Schweitzer, R J Franklin, U Suter, V Taylor (2001) <i>Glia</i> <i></i> <i></i> 36: 1 31-47<br/>

http://publicationslist.org/ueli.suter/refid76 2008-05-24T06:31:48Z

Charcot-Marie-Tooth 1A (CMT1A) neuropathy is caused by duplication of the peripheral myelin protein 22 (PMP22) gene, leading to protein overexpression. Although this protein has a role in regulating Schwann cell growth and peripheral myelin compaction, how altered concentrations of PMP22 impair myelination is unknown. We established dorsal root ganglia (DRG) cultures from a transgenic rat overexpr...<br/><br/>L Nobbio, G Mancardi, M Grandis, G Levi, U Suter, K A Nave, A J Windebank, M Abbruzzese, A Schenone (2001) <i>Ann Neurol</i> <i></i> <i></i> 50: 1 47-55<br/>

http://publicationslist.org/ueli.suter/refid74 2008-05-24T06:31:48Z

The transcription factor Sox10 is required for proper development of various neural crest-derived cell types. Several lineages including melanocytes, autonomic and enteric neurons, and all subtypes of peripheral glia are missing in mice homozygous for Sox10 mutations. Moreover, haploinsufficiency of Sox10 results in neural crest defects that cause Waardenburg/Hirschsprung disease in humans. We pro...<br/><br/>C Paratore, D E Goerich, U Suter, M Wegner, L Sommer (2001) <i>Development</i> <i></i> <i></i> 128: 20 3949-3961<br/>

http://publicationslist.org/ueli.suter/refid70 2008-05-24T06:31:48Z

Neurons regulate Schwann cell proliferation, but little is known about the molecular basis of this interaction. We have examined the possibility that cyclin D1 is a key regulator of the cell cycle in Schwann cells. Myelinating Schwann cells express cyclin D1 in the perinuclear region, but after axons are severed, cyclin D1 is strongly upregulated in parallel with Schwann cell proliferation and tra...<br/><br/>S Atanasoski, S Shumas, C Dickson, S S Scherer, U Suter (2001) <i>Mol Cell Neurosci</i> <i></i> <i></i> 18: 6 581-592<br/>

http://publicationslist.org/ueli.suter/refid72 2008-05-24T06:31:48Z

Until 10 years ago, the genetic basis of Charcot-Marie-Tooth (CMT) disease was largely unknown. With the finding of an intrachromosomal duplication on chromosome 17 in 1991, associated with the most commonly found subtype CMT1A, and the discovery of a point mutation in the peripheral myelin protein-22 (pmp22) gene in the Trembler mouse in 1992, the groundwork was laid down for a novel chapter in t...<br/><br/>P Young, U Suter (2001) <i>Brain Res Brain Res Rev</i> <i></i> <i></i> 36: 2-3 213-221<br/>

http://publicationslist.org/ueli.suter/refid73 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease type 1A (CMT1A) is caused by an increased dosage of the peripheral myelin protein 22 (PMP22) gene or by point mutations affecting the same gene. Based on in vitro data, PMP22 might be involved, besides in its proven role in the regulation of myelination and myelin maintenance, in the control of Schwann cell proliferation and programmed cell death. In this report, we hav...<br/><br/>S Sancho, P Young, U Suter (2001) <i>Brain</i> <i></i> <i></i> 124: Pt 11 2177-2187<br/>

http://publicationslist.org/ueli.suter/refid87 2008-05-24T06:31:48Z

The purpose of this study was to further characterize neuromyotonia in mice with deletions and point mutations of myelin protein genes. Clinical observation showed irregular stretching of the hindlimbs, tremor and generalized myokymia in mice with targeted deletions of the genes encoding myelin protein zero (P0-/-) or peripheral myelin protein 22 (Pmp22-/-), and Trembler mice, which carry a point ...<br/><br/>J Zielasek, R Martini, U Suter, K V Toyka (2000) <i>Muscle Nerve</i> <i></i> <i></i> 23: 5 696-701<br/>

http://publicationslist.org/ueli.suter/refid86 2008-05-24T06:31:48Z

We have generated previously transgenic rats that overexpress peripheral myelin protein 22 (PMP22) in Schwann cells. In the nerves of these animals, Schwann cells have segregated with axons to the normal 1:1 ratio but remain arrested at the promyelinating stage, apparently unable to elaborate myelin sheaths. We have examined gene expression of these dysmyelinating Schwann cells using semiquantitat...<br/><br/>S Niemann, M W Sereda, U Suter, I R Griffiths, K A Nave (2000) <i>J Neurosci</i> <i></i> <i></i> 20: 11 4120-4128<br/>

http://publicationslist.org/ueli.suter/refid84 2008-05-24T06:31:48Z

Myelin and lymphocyte protein (MAL) is a putative tetraspan proteolipid that is highly expressed by Schwann cells and oligodendrocytes as a component of compact myelin. Outside of the nervous system, MAL is found in apical membranes of epithelial cells, mainly in the kidney and stomach. Because MAL is associated with glycosphingolipids, it is thought to be involved in the organization, transport, ...<br/><br/>M Frank, S Atanasoski, S Sancho, J P Magyar, T Rülicke, M E Schwab, U Suter (2000) <i>J Neurochem</i> <i></i> <i></i> 75: 5 1927-1939<br/>

http://publicationslist.org/ueli.suter/refid88 2008-05-24T06:31:48Z

To understand the intimate axon-Schwann cell relationship required for the accurate development and regeneration of the peripheral nervous system (PNS), it is important to elucidate the repertoire of growth factors involved in this tightly regulated bi-directional dialogue. We focused on the identification and functional characterization of receptor tyrosine kinases (RTKs) in Schwann cells to gain...<br/><br/>C S Lobsiger, B Schweitzer, V Taylor, U Suter (2000) <i>Glia</i> <i></i> <i></i> 30: 3 290-300<br/>

http://publicationslist.org/ueli.suter/refid85 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is a structural component of compact peripheral nerve myelin and is likely to play a role in the modulation of cell proliferation and cell spreading. Molecular genetics revealed that mutations affecting the PMP22 gene are responsible for the most common forms of hereditary motor and sensory neuropathies in humans. Computer analysis predicts a tetraspan-membrane...<br/><br/>V Taylor, C Zgraggen, R Naef, U Suter (2000) <i>J Neurosci Res</i> <i></i> <i></i> 62: 1 15-27<br/>

http://publicationslist.org/ueli.suter/refid93 2008-05-24T06:31:48Z

The peripheral myelin protein 22 (PMP22) and the epithelial membrane proteins (EMP-1, -2, and -3) comprise a subfamily of small hydrophobic membrane proteins. The putative four-transmembrane domain structure as well as the genomic structure are highly conserved among family members. PMP22 and EMPs are expressed in many tissues, and functions in cell growth, differentiation, and apoptosis have been...<br/><br/>A M Jetten, U Suter (2000) <i>Prog Nucleic Acid Res Mol Biol</i> <i></i> <i></i> 64: 97-129<br/>

http://publicationslist.org/ueli.suter/refid83 2008-05-24T06:31:48Z

The question of how appropriate cell types are generated in correct numbers during development of the peripheral nervous system has become particularly intriguing with the identification of multipotent progenitor cells in postmigratory targets of the neural crest. Recently, we have provided evidence that community effects in response to factors of the TGFbeta family might represent a mechanism to ...<br/><br/>L Hagedorn, J Floris, U Suter, L Sommer (2000) <i>Dev Biol</i> <i></i> <i></i> 228: 1 57-72<br/>

http://publicationslist.org/ueli.suter/refid80 2008-05-24T06:31:48Z

Genetic methodologies have provided powerful means for investigating the cellular and molecular mechanisms of biological functions. Cell-cell and cell-extracellular matrix interactions in particular have been studied in different functional systems with genetically modified animals. In the peripheral and central nervous system, many aspects of specific processes based on such interactions, includi...<br/><br/>I M Mansuy, U Suter (2000) <i>Exp Physiol</i> <i></i> <i></i> 85: 6 661-679<br/>

http://publicationslist.org/ueli.suter/refid82 2008-05-24T06:31:48Z

Missense mutations in the murine peripheral myelin protein-22 gene (Pmp22) underly the neuropathies in the trembler (Tr) and trembler-J (Tr-J) mice and in some humans with Charcot-Marie-Tooth disease. We have generated replication-defective adenoviruses containing epitope-tagged, wild-type-, Tr-, or Tr-J-PMP22 bicistronic with the Lac-Z reporter gene. These viruses were microinjected into the scia...<br/><br/>J Colby, R Nicholson, K M Dickson, W Orfali, R Naef, U Suter, G J Snipes (2000) <i>Neurobiol Dis</i> <i></i> <i></i> 7: 6 Pt B 561-573<br/>

http://publicationslist.org/ueli.suter/refid90 2008-05-24T06:31:48Z

To investigate the possibility that an autoimmune mechanism may play a role in the hereditary neuropathy Charcot-Marie-Tooth type 1A (CMT1A), sera were analysed by Western blot for anti-peripheral myelin protein 22 (PMP22) autoantibodies. These sera were compared with sera from patients with CMT type 2 (CMT2), acquired peripheral neuropathies such as chronic inflammatory demyelinating neuropathy (...<br/><br/>M F Ritz, J Lechner-Scott, R J Scott, P Fuhr, N Malik, B Erne, V Taylor, U Suter, N Schaeren-Wiemers, A J Steck (2000) <i>J Neuroimmunol</i> <i></i> <i></i> 104: 2 155-163<br/>

http://publicationslist.org/ueli.suter/refid89 2008-05-24T06:31:48Z

Distinct glial cell types of the vertebrate peripheral nervous system (PNS) are derived from the neural crest. Here we show that the expression of the Ets domain transcription factor Erm distinguishes satellite glia from Schwann cells beginning early in rat PNS development. In developing dorsal root ganglia (DRG), Erm is present both in presumptive satellite glia and in neurons. In contrast, Erm i...<br/><br/>L Hagedorn, C Paratore, G Brugnoli, J L Baert, N Mercader, U Suter, L Sommer (2000) <i>Dev Biol</i> <i></i> <i></i> 219: 1 44-58<br/>

http://publicationslist.org/ueli.suter/refid98 2008-05-24T06:31:48Z

Peripheral nerves of mutant mice deficient for peripheral myelin protein 22 (PMP22) or connexin32 (Cx32) display similar pathologies as observed in hereditary human peripheral neuropathies. Mice lacking PMP22 develop focal hypermyelination followed by myelin degeneration and axonal atrophy. Cx32-deficient mice form normal myelin initially but develop demyelination and remyelination at older ages. ...<br/><br/>D H Neuberg, S Sancho, U Suter (1999) <i>J Neurosci Res</i> <i></i> <i></i> 58: 5 612-623<br/>

http://publicationslist.org/ueli.suter/refid108 2008-05-24T06:31:48Z

The most common forms of hereditary motor and sensory neuropathies (HMSN) or Charcot-Marie-Tooth disease (CMT) are associated with mutations affecting myelin genes in the peripheral nervous system. A minor subgroup of CMT type 1A (CMT1A) is caused by point mutations in the gene encoding the peripheral myelin protein 22 (PMP22). To study the mechanisms by which these mutations cause the CMT patholo...<br/><br/>R Naef, U Suter (1999) <i>Neurobiol Dis</i> <i></i> <i></i> 6: 1 1-14<br/>

http://publicationslist.org/ueli.suter/refid107 2008-05-24T06:31:48Z

T Liehr, G Kuhlenbäumer, P Wulf, V Taylor, U Suter, C Van Broeckhoven, J R Lupski, U Claussen, B Rautenstrauss (1999) <i>Genomics</i> <i></i> <i></i> 58: 1 106-108<br/>

http://publicationslist.org/ueli.suter/refid109 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is an integral membrane protein that is essential for the normal formation and maintenance of peripheral myelin. Duplications, deletions, or mutations in the PMP22 gene account for a set of dominantly inherited peripheral neuropathies. The heterozygous Trembler-J (TrJ) genotype in mice is similar genetically to a Charcot-Marie-Tooth disease type 1A pedigree in ...<br/><br/>A R Tobler, L Notterpek, R Naef, V Taylor, U Suter, E M Shooter (1999) <i>J Neurosci</i> <i></i> <i></i> 19: 6 2027-2036<br/>

http://publicationslist.org/ueli.suter/refid94 2008-05-24T06:31:48Z

D H Neuberg, U Suter (1999) <i>Adv Exp Med Biol</i> <i></i> <i></i> 468: 227-236<br/>

http://publicationslist.org/ueli.suter/refid187 2008-11-21T08:45:23Z

A partial duplication of chromosome 17 is associated with Charcot-Marie-Tooth disease type 1A (CMT1A), a demyelinating peripheral neuropathy that causes progressive distal muscle atrophy and sensory impairment. Trisomic expression of peripheral myelin protein 22 (PMP22) whose gene is contained within the duplicated region is considered to be responsible for the disease. By using recombinant gene t...<br/><br/>S Sancho, J P Magyar, A Aguzzi, U Suter (1999) <i>Brain</i> 122 ( Pt 8): 1563-1577<br/>

http://publicationslist.org/ueli.suter/refid103 2008-05-24T06:31:48Z

Tyramide signal amplification has successfully been applied to enhance detection limits of both immunological reactions and in situ hybridization methods. The technique uses short-range deposition of activated tyramide mediated by horseradish peroxidase. We have adapted this method to fluorescence in situ hybridization on embryonic tissue sections using fluorophore-labeled tyramide. The sensitivit...<br/><br/>C Paratore, U Suter, L Sommer (1999) <i>Histochem Cell Biol</i> <i></i> <i></i> 111: 6 435-443<br/>

http://publicationslist.org/ueli.suter/refid110 2008-05-24T06:31:48Z

Mutations in the gene encoding for the myelinating Schwann cell protein P0 have been linked to inherited peripheral neuropathies, including the Charcot-Marie-Tooth type 1B disease (CMT1B) and Dejerine-Sottas syndrome (DSS). Recently generated mice deficient in the P0 gene (P0-/- mice) resemble cases of CMT1B and DSS with impaired myelin dosage (Martini et al., 1995a). Potential approaches to treat...<br/><br/>V Guénard, B Schweitzer, E Flechsig, S Hemmi, R Martini, U Suter, M Schachner (1999) <i>Glia</i> <i></i> <i></i> 25: 2 165-178<br/>

http://publicationslist.org/ueli.suter/refid97 2008-05-24T06:31:48Z

We have generated several PMP22 animal mutants with altered PMP22 gene dosage. A moderate increase in the number of PMP22 genes led to hypomyelination comparable to CMT1A, whereas high copy numbers of transgenic PMP22 resulted in phenotypes resembling more severe forms of hereditary motor and sensory neuropathies. In contrast, eliminating one of the two normal PMP22 genes by gene targeting caused ...<br/><br/>U Suter, K A Nave (1999) <i>Ann N Y Acad Sci</i> <i></i> <i></i> 883: 247-253<br/>

http://publicationslist.org/ueli.suter/refid102 2008-05-24T06:31:48Z

Protein zero (P0) and peripheral myelin protein 22 (PMP22) are most prominently expressed by myelinating Schwann cells as components of compact myelin of the peripheral nervous system (PNS), and mutants affecting P0 and PMP22 show severe defects in myelination. Recent expression studies suggest a role of P0 and PMP22 not only in myelination but also during embryonic development. Here we show that,...<br/><br/>L Hagedorn, U Suter, L Sommer (1999) <i>Development</i> <i></i> <i></i> 126: 17 3781-3794<br/>

http://publicationslist.org/ueli.suter/refid96 2008-05-24T06:31:48Z

We have generated a transgenic rat model of Charcot-Marie-Tooth disease type 1A (CMT1A) providing formal proof that this neuropathy can be caused by increased expression of peripheral myelin protein-22 (PMP22). Heterozygous PMP22-transgenic rats develop muscle weakness and gait abnormalities as well as reduced nerve conduction velocities and EMG abnormalities, which closely resemble recordings in ...<br/><br/>S Niemann, M W Sereda, M Rossner, H Stewart, U Suter, H M Meinck, I R Griffiths, K A Nave (1999) <i>Ann N Y Acad Sci</i> <i></i> <i></i> 883: 254-261<br/>

http://publicationslist.org/ueli.suter/refid99 2008-05-24T06:31:48Z

Peripheral nerves of P0-deficient (P0(-)) mice show a severe dysmyelination and altered expression of several cell surface molecules. In the present study we investigated the subcellular localization of the peripheral myelin protein (PMP)22 in the abnormal axon-Schwann cell units of the mutants. We show by postembedding immunoelectron microscopy that PMP22 is expressed in both noncompacted and abn...<br/><br/>S Carenini, D Neuberg, M Schachner, U Suter, R Martini (1999) <i>Glia</i> <i></i> <i></i> 28: 3 256-264<br/>

http://publicationslist.org/ueli.suter/refid101 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is a component of compact myelin of the peripheral nervous system (PNS). Mutations affecting PMP22 are associated with hereditary neuropathies in humans and rodents. Although mammalian PMP22 is expressed in several tissues, the disease pathology is restricted to the PNS. We describe the characterization of a PMP22-related cDNA from zebrafish and the distributio...<br/><br/>P Wulf, R R Bernhardt, U Suter (1999) <i>J Neurosci Res</i> <i></i> <i></i> 57: 4 467-478<br/>

http://publicationslist.org/ueli.suter/refid100 2008-05-24T06:31:48Z

Epithelial membrane protein 1 (EMP1) is a member of the peripheral myelin protein 22 (PMP22) family. This family is best known for the crucial contribution of PMP22 to the development and maintenance of the peripheral nervous system (PNS). PMP22 is widely expressed, with highest levels in myelinating Schwann cells, and mutations affecting the PMP22 gene lead to PNS-restricted neuropathies. We have...<br/><br/>P Wulf, U Suter (1999) <i>Brain Res Dev Brain Res</i> <i></i> <i></i> 116: 2 169-180<br/>

http://publicationslist.org/ueli.suter/refid116 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is a small, hydrophobic glycoprotein, which is most prominently expressed by Schwann cells as a component of compact myelin of the peripheral nervous system (PNS). Recent progress in molecular genetics revealed that mutations affecting the PMP22 gene including duplications, deletions, and point mutations are responsible for the most common forms of hereditary p...<br/><br/>R Naef, U Suter (1998) <i>Microsc Res Tech</i> <i></i> <i></i> 41: 5 359-371<br/>

http://publicationslist.org/ueli.suter/refid117 2008-05-24T06:31:48Z

Recent data show that during development protein zero, P0, the most abundant peripheral nerve myelin protein, is detecable long before myelination. In particular, the expression of P0 in a fraction of migrating neural crest reveals progenitor cell heterogeneity in the developing PNS. Here we review the regulation and potential function of P0 during peripheral gliogenesis.<br/><br/>L Sommer, U Suter (1998) <i>Cell Tissue Res</i> <i></i> <i></i> 292: 1 11-16<br/>

http://publicationslist.org/ueli.suter/refid115 2008-05-24T06:31:48Z

We have identified and isolated a cDNA that codes for the novel protein NMP35 (neural membrane protein 35) using RNA arbitrarily primed PCR (RAP-PCR) for differential display in the developing rat sciatic nerve. NMP35 is predominantly found in the adult nervous system where both transcripts and protein are strongly upregulated during postnatal development. In situ hybridization studies show that N...<br/><br/>B Schweitzer, V Taylor, A A Welcher, M McClelland, U Suter (1998) <i>Mol Cell Neurosci</i> <i></i> <i></i> 11: 5-6 260-273<br/>

http://publicationslist.org/ueli.suter/refid114 2008-05-24T06:31:48Z

Mutant mice that lack either protein zero (P0) or connexin 32 (Cx32) were generated previously to investigate the function of these myelin proteins in peripheral nerves and to assess the value of these mice as animal models for hereditary human peripheral neuropathies. Mice that are completely devoid of P0 expression (P0(+/0)) show a complex phenotype that is characterized by hypomyelination, comp...<br/><br/>D H Neuberg, S Carenini, M Schachner, R Martini, U Suter (1998) <i>J Neurosci Res</i> <i></i> <i></i> 53: 5 542-550<br/>

http://publicationslist.org/ueli.suter/refid113 2008-05-24T06:31:48Z

To understand better the mechanisms by which progesterone (PROG) promotes myelination in the PNS, cultured rat Schwann cells were transiently transfected with reporter constructs in which luciferase expression was controlled by the promoter region of either the peripheral myelin protein-22 (PMP22) or the protein zero (P0) genes. PROG stimulated the P0 promoter and promoter 1, but not promoter 2, o...<br/><br/>F Désarnaud, A N Do Thi, A M Brown, G Lemke, U Suter, E E Baulieu, M Schumacher (1998) <i>J Neurochem</i> <i></i> <i></i> 71: 4 1765-1768<br/>

http://publicationslist.org/ueli.suter/refid131 2008-05-24T06:31:48Z

V Taylor, U Suter (1997) <i>Prog Nucleic Acid Res Mol Biol</i> <i></i> <i></i> 56: 225-256<br/>

http://publicationslist.org/ueli.suter/refid130 2008-05-24T06:31:48Z

The naturally occurring mouse mutant Trembler (Tr) represents an animal model for inherited human neuropathies caused by point mutations affecting peripheral myelin protein 22 (PMP22). We describe the likely pathogenic cellular mechanism underlying the observed myelin deficiency. In Tr/+ animals, PMP22 immunoreactivity was found not only in compact myelin but also abundantly in the cytoplasm of Sc...<br/><br/>R Naef, K Adlkofer, B Lescher, U Suter (1997) <i>Mol Cell Neurosci</i> <i></i> <i></i> 9: 1 13-25<br/>

http://publicationslist.org/ueli.suter/refid133 2008-05-24T06:31:48Z

The recently determined structure of protein zero, P0, the most abundant peripheral nerve myelin protein, provides new insights into the molecular structure of myelin and potential disease mechanisms in hereditary peripheral neuropathies.<br/><br/>U Suter (1997) <i>Curr Biol</i> <i></i> <i></i> 7: 1 R21-R23<br/>

http://publicationslist.org/ueli.suter/refid129 2008-05-24T06:31:48Z

The First Workshop of the European Consortium on Charcot-Marie-Tooth (CMT) disease brought together neuroscientists, molecular and cell biologists, neuropathologists, neurologists, and geneticists with a common interest in the understanding of the fundamental mechanisms that underlie the pathogenesis of CMT. The interdisciplinary group of 25 expert scientists discussed recent advances in (i) molec...<br/><br/>H W Müller, U Suter, C Van Broeckhoven, C O Hanemann, E Nelis, V Timmerman, S Sancho, L Barrio, P Bolhuis, R Dermietzel, M Frank, A Gabreëls-Festen, C Gillen, N Haites, G Levi, E Mariman, R Martini, K Nave, B Rautenstrauss, M Schachner, A Schenone, C Schneider, M Schröder, K Willecke, O Haneman (1997) <i>Neurobiol Dis</i> <i></i> <i></i> 4: 3-4 215-220<br/>

http://publicationslist.org/ueli.suter/refid132 2008-05-24T06:31:48Z

G J Snipes, U Suter (1997) <i>Subcell Biochem</i> <i></i> <i></i> 28: 173-204<br/>

http://publicationslist.org/ueli.suter/refid122 2008-05-24T06:31:48Z

Schwann cells express low levels of myelin proteins in the absence of neurons. When Schwann cells and neurons are cultured together the production of myelin proteins is elevated, and myelin is formed. For peripheral myelin protein 22 (PMP22), the exact amount of protein produced is critical, because peripheral neuropathies result from its underexpression or overexpression. In this study we examine...<br/><br/>S Pareek, L Notterpek, G J Snipes, R Naef, W Sossin, J Laliberté, S Iacampo, U Suter, E M Shooter, R A Murphy (1997) <i>J Neurosci</i> <i></i> <i></i> 17: 20 7754-7762<br/>

http://publicationslist.org/ueli.suter/refid128 2008-05-24T06:31:48Z

An increasing number of four-transmembrane proteins has been found to be associated with CNS and PNS myelin. Some of these proteins play crucial roles in the development and maintenance of the nervous system. In the CNS, proteolipid protein (PLP) is mutated in the myelin disorder Pelizaeus-Merzbacher disease and in spastic paraplegia, while in the PNS, peripheral myelin protein 22 (PMP22) and conn...<br/><br/>J P Magyar, C Ebensperger, N Schaeren-Wiemers, U Suter (1997) <i>Gene</i> <i></i> <i></i> 189: 2 269-275<br/>

http://publicationslist.org/ueli.suter/refid121 2008-05-24T06:31:48Z

The most common form of Charcot-Marie-Tooth disease, CMT1A, is correlated with a 1.5 megabase duplication on chromosome 17p.11.2 containing the peripheral myelin protein 22 (PMP22) gene. Deletion of the same region is associated with a second inherited neural disorder, the hereditary neuropathy with liability to pressure palsies (HNPP). Moreover, several distinct point mutations within the PMP22 c...<br/><br/>K Adlkofer, R Naef, U Suter (1997) <i>J Neurosci Res</i> <i></i> <i></i> 49: 6 671-680<br/>

http://publicationslist.org/ueli.suter/refid119 2008-05-24T06:31:48Z

K V Toyka, J Zielasek, K Ricker, K Adlkofer, U Suter (1997) <i>J Neurol Neurosurg Psychiatry</i> <i></i> <i></i> 63: 6 812-813<br/>

http://publicationslist.org/ueli.suter/refid127 2008-05-24T06:31:48Z

Mutations affecting the connexin 32 (Cx32) gene are associated with the X-linked form of the hereditary peripheral neuropathy Charcot-Marie-Tooth disease (CMTX). We show that Cx32-deficient mice develop a late-onset progressive peripheral neuropathy with abnormalities comparable to those associated with CMTX, thus providing proof of the critical role of Cx32 in the maintenance of peripheral nerve ...<br/><br/>P Anzini, D H Neuberg, M Schachner, E Nelles, K Willecke, J Zielasek, K V Toyka, U Suter, R Martini (1997) <i>J Neurosci</i> <i></i> <i></i> 17: 12 4545-4551<br/>

http://publicationslist.org/ueli.suter/refid126 2008-05-24T06:31:48Z

Hereditary neuropathy with liability to pressure palsy (HNPP) is associated with a heterozygous 1.5 megabase deletion on chromosome 17 that includes the peripheral myelin protein (PMP) gene PMP22. We show that heterozygous PMP22 knock-out mice, which carry only one functional pmp22 allele and thus genetically mimic HNPP closely, display similar morphological and electrophysiological features as ob...<br/><br/>K Adlkofer, R Frei, D H Neuberg, J Zielasek, K V Toyka, U Suter (1997) <i>J Neurosci</i> <i></i> <i></i> 17: 12 4662-4671<br/>

http://publicationslist.org/ueli.suter/refid142 2008-05-24T06:31:48Z

Peripheral Myelin Protein-22 (PMP22) is a membrane glycoprotein which represents up to 5% of total protein in myelin of peripheral nerves. Mutations affecting the PMP22 gene have been linked to the inherited peripheral neuropathies Charcot-Marie-Tooth disease type 1A (CMT1A; duplications and point mutations), Dejerine-Sottas syndrome (DSS; point mutations), and hereditary neuropathy with liability...<br/><br/>C Haney, G J Snipes, E M Shooter, U Suter, C Garcia, J W Griffin, B D Trapp (1996) <i>J Neuropathol Exp Neurol</i> <i></i> <i></i> 55: 3 290-299<br/>

http://publicationslist.org/ueli.suter/refid143 2008-05-24T06:31:48Z

Protein tyrosine phosphatases (PTP) are increasingly appreciated to play a crucial function in the development, homeostasis and regeneration of the nervous system. In an attempt to determine the set of PTPs that are expressed in the PNS, we have employed polymerase chain reaction (PCR)-based strategies. Degenerate oligonucleotide primers which were designed based on PTP-consensus motifs were used ...<br/><br/>D H Neuberg, J P Magyar, B Schweitzer, U Suter (1996) <i>J Peripher Nerv Syst</i> <i></i> <i></i> 1: 3 231-239<br/>

http://publicationslist.org/ueli.suter/refid140 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy in humans and has been associated with a partial duplication of chromosome 17 (CMT type 1A). We have generated a transgenic rat model of this disease and provide experimental evidence that CMT1A is caused by increased expression of the gene for peripheral myelin protein-22 (PMP22, gas-3). PMP22-transgenic rats develop gait a...<br/><br/>M Sereda, I Griffiths, A Pühlhofer, H Stewart, M J Rossner, F Zimmerman, J P Magyar, A Schneider, E Hund, H M Meinck, U Suter, K A Nave (1996) <i>Neuron</i> <i></i> <i></i> 16: 5 1049-1060<br/>

http://publicationslist.org/ueli.suter/refid138 2008-05-24T06:31:48Z

An intrachromosomal duplication containing the PMP22 gene is associated with the human hereditary peripheral neuropathy Charcot-Marie-Tooth disease type 1A, and PMP22 overexpression as a consequence of increased PMP22 gene dosage has been suggested as causative event in this frequent disorder of peripheral nerves. We have generated transgenic mice that carry additional copies of the pmp22 gene to ...<br/><br/>J P Magyar, R Martini, T Ruelicke, A Aguzzi, K Adlkofer, Z Dembic, J Zielasek, K V Toyka, U Suter (1996) <i>J Neurosci</i> <i></i> <i></i> 16: 17 5351-5360<br/>

http://publicationslist.org/ueli.suter/refid137 2008-05-24T06:31:48Z

The PMP22/EMP/MP20 gene family includes four closely related proteins, peripheral myelin protein-22 (PMP22), epithelial membrane protein-1 (EMP-1), epithelial membrane protein-2 (EMP-2), and epithelial membrane protein-3 (EMP-3), which share amino acid identities ranging from 33 to 43%. In addition, the lens-specific membrane protein MP20 represents a more distant relative. Functionally, this fami...<br/><br/>C S Lobsiger, J P Magyar, V Taylor, P Wulf, A A Welcher, A E Program, U Suter (1996) <i>Genomics</i> <i></i> <i></i> 36: 3 379-387<br/>

http://publicationslist.org/ueli.suter/refid135 2008-05-24T06:31:48Z

The varied roles that neurotrophins play in the development and activity-dependent plasticity of the nervous system presumably require that the sites and quantity of neurotrophin release be precisely regulated. As a step toward understanding how different neurotrophins are sorted and secreted by neurons, we expressed nerve growth factor (NGF), brain-derived neurotrophic factor, and neurotrophin-3 ...<br/><br/>J V Heymach, A Krüttgen, U Suter, E M Shooter (1996) <i>J Biol Chem</i> <i></i> <i></i> 271: 41 25430-25437<br/>

http://publicationslist.org/ueli.suter/refid136 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is expressed by Schwann cells in the peripheral nervous system (PNS), and mutations affecting the PMP22 gene are associated with hereditary motor and sensory neuropathies. We have previously defined the PMP22/EMP/MP20 gene family by characterizing the PMP22-related epithelial membrane protein-1 (EMP-1). We now report the identification of two additional members...<br/><br/>V Taylor, U Suter (1996) <i>Gene</i> <i></i> <i></i> 175: 1-2 115-120<br/>

http://publicationslist.org/ueli.suter/refid150 2008-05-24T06:31:48Z

U Suter, G J Snipes (1995) <i>Annu Rev Neurosci</i> <i></i> <i></i> 18: 45-75<br/>

http://publicationslist.org/ueli.suter/refid151 2008-05-24T06:31:48Z

G J Snipes, U Suter, A A Welcher, E M Shooter (1995) <i>Prog Brain Res</i> <i></i> <i></i> 105: 319-325<br/>

http://publicationslist.org/ueli.suter/refid149 2008-05-24T06:31:48Z

Mutations affecting the peripheral myelin protein 22 (PMP22) gene are associated with inherited motor and sensory neuropathies in mouse (Trembler and Trembler-J) and human (Charcot-Marie-Tooth disease type 1A and Dejerine-Sottas syndrome). Although genetic studies have established a critical role of PMP22 in the formation and/or maintenance of myelin in the peripheral nervous system, the biologica...<br/><br/>U Suter, G J Snipes (1995) <i>J Neurosci Res</i> <i></i> <i></i> 40: 2 145-151<br/>

http://publicationslist.org/ueli.suter/refid146 2008-05-24T06:31:48Z

Peripheral myelin protein PMP22 has been suggested to have a role in peripheral nerve myelination and cell proliferation. Defects at the PMP22 locus are associated with peripheral neuropathies such as Charcot-Marie-Tooth disease type 1A. We now demonstrate that mice devoid of Pmp22 are retarded in the onset of myelination and develop abundant sausage-like hypermyelination structures (tomacula) at ...<br/><br/>K Adlkofer, R Martini, A Aguzzi, J Zielasek, K V Toyka, U Suter (1995) <i>Nat Genet</i> <i></i> <i></i> 11: 3 274-280<br/>

http://publicationslist.org/ueli.suter/refid144 2008-05-24T06:31:48Z

The gene encoding the peripheral myelin protein PMP22 is affected by various mutations in the hereditary peripheral neuropathies Charcot-Marie-Tooth disease type 1A (CMT1A), Déjérine-Sottas syndrome (DSS) and hereditary neuropathy with liability to pressure palsies (HNPP). In contrast to the recent remarkable progress in the genetics of the PMP22 gene, the biological function of PMP22 remains la...<br/><br/>D Baechner, T Liehr, H Hameister, H Altenberger, H Grehl, U Suter, B Rautenstrauss (1995) <i>J Neurosci Res</i> <i></i> <i></i> 42: 6 733-741<br/>

http://publicationslist.org/ueli.suter/refid148 2008-05-24T06:31:48Z

Myelin contains a number of proteins, the major examples of which are protein zero (Po), P2 protein, peripheral myelin protein 22 (PMP22), myelin basic proteins (MBPs), myelin-associated glycoprotein (MAG) and the recently described connexin 32 (Cx32). This list is probably still incomplete. The localisation and possible functions of these proteins are reviewed. In the past few years a number of i...<br/><br/>G J Snipes, U Suter (1995) <i>J Anat</i> <i></i> <i></i> 186 ( Pt 3): 483-494<br/>

http://publicationslist.org/ueli.suter/refid145 2008-05-24T06:31:48Z

Peripheral myelin protein 22 (PMP22) is expressed in many tissues but mainly by Schwann cells as a component of compact myelin of the peripheral nervous system (PNS). Mutations affecting PMP22 are associated with hereditary motor and sensory neuropathies. Although these phenotypes are restricted to the PNS, PMP22 is thought to play a dual role in myelin formation and in cell proliferation. We desc...<br/><br/>V Taylor, A A Welcher, A E Program, U Suter (1995) <i>J Biol Chem</i> <i></i> <i></i> 270: 48 28824-28833<br/>

http://publicationslist.org/ueli.suter/refid147 2008-05-24T06:31:48Z

The Hereditary Motor and Sensory Neuropathies (HMSNs) are well known to be clinically, morphologically, and genetically heterogeneous. Yet, recent advances in the cellular and molecular biology of the peripheral nervous system coupled with remarkable progress in human and mouse genetics have provided a framework that has profoundly changed our understanding of the pathogenesis of these diseases. I...<br/><br/>G J Snipes, U Suter (1995) <i>Brain Pathol</i> <i></i> <i></i> 5: 3 233-247<br/>

http://publicationslist.org/ueli.suter/refid154 2008-05-24T06:31:48Z

Progress in the elucidation of the genetic basis for inherited peripheral neuropathies has been remarkable over the last years. In particular, the molecular mechanisms underlying the autosomal dominantly inherited disorders Charcot-Marie-Tooth disease type 1A (CMT1A), Charcot-Marie-Tooth disease type 1B (CMT1B), and hereditary neuropathy with liability to pressure palsies (HNPP) have been determin...<br/><br/>U Suter, P I Patel (1994) <i>Hum Mutat</i> <i></i> <i></i> 3: 2 95-102<br/>

http://publicationslist.org/ueli.suter/refid153 2008-05-24T06:31:48Z

G J Snipes, U Suter (1994) <i>Trends Neurosci</i> <i></i> <i></i> 17: 10 399-401<br/>

http://publicationslist.org/ueli.suter/refid152 2008-05-24T06:31:48Z

Mutations affecting the peripheral myelin protein-22 (PMP22) gene have been shown to be associated with inherited peripheral neuropathies. To provide the molecular basis for the analysis of such mutations, we have cloned and characterized the human PMP22 gene. It spans approximately 40 kilobases and contains four coding exons. Detailed analysis of its 5'-flanking region suggested the presence of t...<br/><br/>U Suter, G J Snipes, R Schoener-Scott, A A Welcher, S Pareek, J R Lupski, R A Murphy, E M Shooter, P I Patel (1994) <i>J Biol Chem</i> <i></i> <i></i> 269: 41 25795-25808<br/>

http://publicationslist.org/ueli.suter/refid160 2008-05-24T06:31:48Z

Peripheral myelin protein, 22 kDa (PMP22), is a myelin molecule associated with Schwann cells in peripheral nerves (Snipes, G. J., Suter, U., Welcher, A. A., and Shooter, E. M. (1992) J. Cell Biol. 117, 225-238). Mutations affecting the PMP22 gene have been implicated in the trembler mutation in mice (Suter, U., Welcher, A. A., Ozcelik, T., Snipes, G. J., Kosaras, B., Francke, U., Billings-Gagliar...<br/><br/>S Pareek, U Suter, G J Snipes, A A Welcher, E M Shooter, R A Murphy (1993) <i>J Biol Chem</i> <i></i> <i></i> 268: 14 10372-10379<br/>

http://publicationslist.org/ueli.suter/refid161 2008-05-24T06:31:48Z

Since the first description of the autosomal dominant inherited peripheral neuropathy Charcot-Marie-Tooth (CMT) disease over a century ago, there has been considerable disagreement, based on morphological abnormalities of both the axons of peripheral nerves and their surrounding Schwann cells, as to whether this disorder is due primarily to an autonomous Schwann cell defect or an autonomous neuron...<br/><br/>U Suter, A A Welcher, G J Snipes (1993) <i>Trends Neurosci</i> <i></i> <i></i> 16: 2 50-56<br/>

http://publicationslist.org/ueli.suter/refid159 2008-05-24T06:31:48Z

BACKGROUND. Charcot-Marie-Tooth disease (CMT) is the most common inherited peripheral neuropathy. CMT type 1A is associated with a 1.5-megabase DNA duplication in region p11.2-p12 of chromosome 17 in most patients. An increased dosage of a gene within the duplicated segment appears to cause the disease. The PMP22 gene, which encodes a myelin protein, has been mapped within the duplication and prop...<br/><br/>B B Roa, C A Garcia, U Suter, D A Kulpa, C A Wise, J Mueller, A A Welcher, G J Snipes, E M Shooter, P I Patel, J R Lupski (1993) <i>N Engl J Med</i> <i></i> <i></i> 329: 2 96-101<br/>

http://publicationslist.org/ueli.suter/refid156 2008-05-24T06:31:48Z

Molecular genetic studies have established that mutations in the gene encoding the 22-kDa peripheral myelin protein (PMP-22) are responsible for hereditary peripheral neuropathies in the trembler mouse and in a subset of humans with Charcot-Marie-Tooth disease, type 1a. The function of the PMP-22 protein remains unknown. Several studies on myelin proteins in the PNS have indicated that the L2/HNK-...<br/><br/>G J Snipes, U Suter, E M Shooter (1993) <i>J Neurochem</i> <i></i> <i></i> 61: 5 1961-1964<br/>

http://publicationslist.org/ueli.suter/refid158 2008-05-24T06:31:48Z

Myelin formation and maintenance requires complex interactions between neurons and glia, and between the integral protein and lipid components of the myelin sheath. Many of the underlying mechanisms may be examined by studying the perturbations caused by spontaneous and targeted mutations in myelin protein genes. This review summarizes the progress in our understanding of these mutations with an e...<br/><br/>G J Snipes, U Suter, E M Shooter (1993) <i>Curr Opin Neurobiol</i> <i></i> <i></i> 3: 5 694-702<br/>

http://publicationslist.org/ueli.suter/refid155 2008-05-24T06:31:48Z

A series of mouse nerve growth factor (NGF) deletion mutants have been constructed using in vitro mutagenesis to define domains of the protein essential for its activity. Deletions of the amino or carboxyl termini of mature NGF or of an internal domain, which normally produces a surface-exposed reverse turn, have been analyzed. Mutants with deletions in the amino terminus or in the reverse turn re...<br/><br/>C C Drinkwater, P A Barker, U Suter, E M Shooter (1993) <i>J Biol Chem</i> <i></i> <i></i> 268: 31 23202-23207<br/>

http://publicationslist.org/ueli.suter/refid157 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease type 1A (CMT1A) is an autosomal dominant neuropathy that can be caused by dominant point mutations in PMP22 which encodes a peripheral nerve myelin protein. Usually, CMT1A is caused by the duplication of a 1.5-megabase (Mb) region on chromosome 17p11.2-p12 containing PMP22. Deletion of a similar 1.5-Mb region is associated with hereditary neuropathy with liability to pr...<br/><br/>B B Roa, C A Garcia, L Pentao, J M Killian, B J Trask, U Suter, G J Snipes, R Ortiz-Lopez, E M Shooter, P I Patel, J R Lupski (1993) <i>Nat Genet</i> <i></i> <i></i> 5: 2 189-194<br/>

http://publicationslist.org/ueli.suter/refid167 2008-05-24T06:31:48Z

Despite their extensive sequence identities at the amino acid level (approximately 55%), NGF and brain-derived neurotrophic factor (BDNF) display distinct neuronal specificity toward neurons of both the PNS and CNS. To explore which region(s) within these neurotrophic factors might determine their differential actions on various subpopulations of peripheral neurons, a systematic series (homolog-sc...<br/><br/>U Suter, C Angst, C L Tien, C C Drinkwater, R M Lindsay, E M Shooter (1992) <i>J Neurosci</i> <i></i> <i></i> 12: 1 306-318<br/>

http://publicationslist.org/ueli.suter/refid168 2008-05-24T06:31:48Z

A A Welcher, M De Leon, U Suter, G J Snipes, S O Meakin, E M Shooter (1992) <i>Prog Brain Res</i> <i></i> <i></i> 94: 163-176<br/>

http://publicationslist.org/ueli.suter/refid166 2008-05-24T06:31:48Z

Two distinct nerve growth factor receptor (NGFR) complexes are present on NGF-responsive cell types; these correspond to 100 kDa and 158 kDa for the fast (fNGFR) and the slow (sNGFR) NGFRs, respectively. Previous studies indicate that each complex is derived from a separate gene product and that the sNGFR contains tyrosine kinase activity. The cDNA encoding the fNGFR has previously been cloned. In...<br/><br/>S O Meakin, U Suter, C C Drinkwater, A A Welcher, E M Shooter (1992) <i>Proc Natl Acad Sci U S A</i> <i></i> <i></i> 89: 6 2374-2378<br/>

http://publicationslist.org/ueli.suter/refid165 2008-05-24T06:31:48Z

The autosomal dominant trembler mutation (Tr), maps to mouse chromosome 11 (ref. 2) and manifests as a Schwann-cell defect characterized by severe hypomyelination and continuing Schwann-cell proliferation throughout life. Affected animals move clumsily and develop tremor and transient seizures at a young age. We have recently described a potentially growth-regulating myelin protein, peripheral mye...<br/><br/>U Suter, A A Welcher, T Ozcelik, G J Snipes, B Kosaras, U Francke, S Billings-Gagliardi, R L Sidman, E M Shooter (1992) <i>Nature</i> <i></i> <i></i> 356: 6366 241-244<br/>

http://publicationslist.org/ueli.suter/refid162 2008-05-24T06:31:48Z

Charcot-Marie-Tooth disease type 1A (CMT1A) is an autosomal dominant peripheral neuropathy associated with a large DNA duplication on the short arm of human chromosome 17. The trembler (Tr) mouse serves as a model for CMT1A because of phenotypic similarities and because the Tr locus maps to mouse chromosome 11 in a region of conserved synteny with human chromosome 17. Recently, the peripheral myel...<br/><br/>P I Patel, B B Roa, A A Welcher, R Schoener-Scott, B J Trask, L Pentao, G J Snipes, C A Garcia, U Francke, E M Shooter, J R Lupski, U Suter (1992) <i>Nat Genet</i> <i></i> <i></i> 1: 3 159-165<br/>

http://publicationslist.org/ueli.suter/refid163 2008-05-24T06:31:48Z

Peripheral myelin protein PMP-22 is a potential growth-regulating myelin protein that is expressed by Schwann cells and predominantly localized in compact peripheral myelin. A point mutation in the Pmp-22 gene of inbred trembler (Tr) mice was identified and proposed to be responsible for the Tr phenotype, which is characterized by paralysis of the limbs as well as tremors and transient seizures. I...<br/><br/>U Suter, J J Moskow, A A Welcher, G J Snipes, B Kosaras, R L Sidman, A M Buchberg, E M Shooter (1992) <i>Proc Natl Acad Sci U S A</i> <i></i> <i></i> 89: 10 4382-4386<br/>

http://publicationslist.org/ueli.suter/refid164 2008-05-24T06:31:48Z

We have recently described a novel cDNA, SR13 (Welcher, A. A., U. Suter, M. De Leon, G. J. Snipes, and E. M. Shooter. 1991. Proc. Natl. Acad. Sci. USA. 88:7195-7199), that is repressed after sciatic nerve crush injury and shows homology to both the growth arrest-specific mRNA, gas3 (Manfioletti, G., M. E. Ruaro, G. Del Sal, L. Philipson, and C. Schneider, 1990. Mol. Cell Biol. 10:2924-2930), and t...<br/><br/>G J Snipes, U Suter, A A Welcher, E M Shooter (1992) <i>J Cell Biol</i> <i></i> <i></i> 117: 1 225-238<br/>

http://publicationslist.org/ueli.suter/refid176 2008-05-24T06:31:48Z

G Delespesse, U Suter, D Mossalayi, B Bettler, M Sarfati, H Hofstetter, E Kilcherr, P Debre, A Dalloul (1991) <i>Adv Immunol</i> <i></i> <i></i> 49: 149-191<br/>

http://publicationslist.org/ueli.suter/refid174 2008-05-24T06:31:48Z

Current research into regeneration of the nervous system has focused on defining the molecular events that occur during regeneration. One well-characterized system for studying nerve regeneration is the sciatic nerve of rat. Numerous studies have characterized the sequence of events that occur after a crush injury to the sciatic nerve (Cajal 1928; Hall 1989). These events include axon and myelin b...<br/><br/>A A Welcher, U Suter, M De Leon, C M Bitler, E M Shooter (1991) <i>Philos Trans R Soc Lond B Biol Sci</i> <i></i> <i></i> 331: 1261 295-301<br/>

http://publicationslist.org/ueli.suter/refid175 2008-05-24T06:31:48Z

We recently reported that the sera of chronic lymphocytic leukemia (CLL) patients contained 3-500 times more soluble CD23 (or IgE-BF) than the sera of patients with other lymphoproliferative diseases or normal individuals and that their B cells (B-CLLs) overexpressed CD23 Ag. In the present report, we extended these studies and showed that CD5+ B cells from all CLL patients (n = 15) co-express CD2...<br/><br/>S Fournier, I D Tran, U Suter, G Biron, G Delespesse, M Sarfati (1991) <i>Leuk Res</i> <i></i> <i></i> 15: 7 609-618<br/>

http://publicationslist.org/ueli.suter/refid169 2008-05-24T06:31:48Z

By using in vitro DNA mutagenesis, we replaced the tryptophan residue at position 21 in mouse nerve growth factor (NGF) with either phenylalanine, leucine or serine. Yield, biological activity, immunological reactivity and receptor binding of the recombinant proteins were determined. All three mutants were produced at considerably lower yields than wild-type NGF, with the serine mutant being undet...<br/><br/>C C Drinkwater, U Suter, C Angst, E M Shooter (1991) <i>Proc Biol Sci</i> <i></i> <i></i> 246: 1317 307-313<br/>

http://publicationslist.org/ueli.suter/refid170 2008-05-24T06:31:48Z

The three members of the neurotrophin family (NGF, BDNF and NT-3) are synthesized as large precursor proteins which undergo proteolytic processing to yield biologically active, mature neurotrophic factors. We have used in vitro mutagenesis to examine the pro-region in the NGF precursor protein as a first step towards a general understanding of the role of propeptides in the biosynthesis of neurotr...<br/><br/>U Suter, J V Heymach, E M Shooter (1991) <i>EMBO J</i> <i></i> <i></i> 10: 9 2395-2400<br/>

http://publicationslist.org/ueli.suter/refid171 2008-05-24T06:31:48Z

Striking features of the cellular response to sciatic nerve injury are the proliferation of Schwann cells in the distal nerve stump and the downregulation of myelin-specific gene expression. Once the axons regrow, the Schwann cells differentiate again to reform the myelin sheaths. We have isolated a rat cDNA, SR13, which is strongly downregulated in the initial phase after sciatic nerve injury. Th...<br/><br/>A A Welcher, U Suter, M De Leon, G J Snipes, E M Shooter (1991) <i>Proc Natl Acad Sci U S A</i> <i></i> <i></i> 88: 16 7195-7199<br/>

http://publicationslist.org/ueli.suter/refid172 2008-05-24T06:31:48Z

Mammalian peripheral nerve fibres can regenerate after injury. In an attempt toward a better understanding of the underlying molecular events, we have isolated novel and known rat cDNA sequences, the expression of which are regulated during sciatic nerve regeneration. For this purpose, cDNA libraries were constructed from either the nerve segment distal to the crush site or the corresponding contr...<br/><br/>M De Leon, A A Welcher, U Suter, E M Shooter (1991) <i>J Neurosci Res</i> <i></i> <i></i> 29: 4 437-448<br/>

http://publicationslist.org/ueli.suter/refid179 2008-05-24T06:31:48Z

The low-affinity receptor for IgE (Fc epsilon R II) is mainly expressed on B lymphocytes, although it may also be found on some monocytes, eosinophils, and platelets. The presence of Fc epsilon R II on T cells is still controversial, but our results demonstrate that it is expressed on some HTLV-I-transformed T lymphocytes, and they strongly suggest that it may be found on a small proportion of nor...<br/><br/>G Delespesse, M Sarfati, H Hofstetter, H Frost, E Kilchherr, U Suter (1989) <i>Int Arch Allergy Appl Immunol</i> <i></i> <i></i> 88: 1-2 18-22<br/>

http://publicationslist.org/ueli.suter/refid177 2008-05-24T06:31:48Z

Two species, Fc epsilon RIIa and Fc epsilon RIIb, of the human low-affinity receptor for IgE (Fc epsilon RII/CD23) have recently been described. They differ by only six amino acids in the cytoplasmic N-terminus and are generated by different cell-specific transcriptional start sites that lead to distinct 5'-leader sequences in the corresponding mRNA. In this study, we present the analysis of the p...<br/><br/>U Suter, G Texido, H Hofstetter (1989) <i>J Immunol</i> <i></i> <i></i> 143: 9 3087-3092<br/>

http://publicationslist.org/ueli.suter/refid178 2008-05-24T06:31:48Z

G Delespesse, H Hofstetter, M Sarfati, U Suter, T Nakajima, H Frost, M Letellier, R Peleman, E Kilchherr (1989) <i>Chem Immunol</i> <i></i> <i></i> 47: 79-105<br/>

http://publicationslist.org/ueli.suter/refid180 2008-05-24T06:31:48Z

G Delespesse, M Sarfati, H Hofstetter, U Suter, T Nakajima, R Peleman, M Letellier, E Kilchherr, H Frost (1988) <i>Immunol Invest</i> <i></i> <i></i> 17: 5 363-387<br/>

http://publicationslist.org/ueli.suter/refid183 2008-05-24T06:31:48Z

Low-affinity receptors (Fc epsilon R) and secreted factors (IgE-BF) which bind to immunoglobulins of the IgE isotype play a key role in the regulation of human IgE synthesis. We report here the cloning of a cDNA coding for the Fc epsilon R of the human B-lymphoblast cell line RPMI 8866. The nucleotide sequence of this cDNA predicts a polypeptide with 321 amino acids and a mol. wt of 36,281 daltons...<br/><br/>C Lüdin, H Hofstetter, M Sarfati, C A Levy, U Suter, D Alaimo, E Kilchherr, H Frost, G Delespesse (1987) <i>EMBO J</i> <i></i> <i></i> 6: 1 109-114<br/>

http://publicationslist.org/ueli.suter/refid181 2008-05-24T06:31:48Z

We previously described human T cell leukemia/lymphoma virus-1-transformed T cell clones expressing surface molecules binding to monoclonal antibodies against lymphocyte Fc epsilon receptor (Fc epsilon R) and releasing soluble factors binding to both IgE and to monoclonal antibody to lymphocyte Fc epsilon R. In this study, one such clone (HE1-11) was tested for the presence of mRNA hybridizing wit...<br/><br/>M Sarfati, T B Nutman, U Suter, H Hofstetter, G Delespesse (1987) <i>J Immunol</i> <i></i> <i></i> 139: 12 4055-4060<br/>

http://publicationslist.org/ueli.suter/refid182 2008-05-24T06:31:48Z

Overlapping clones which contain the complete gene encoding the human lymphocyte IgE receptor (MW:45kd; identical with CD23), were isolated from human genomic lambda-libraries. The gene spans approximately 13kb and comprises 11 exons. The 5'-end of the mRNA was mapped by primer extension and S1-mapping, revealing two initiation sites for transcription. Two corresponding TATA boxes were identified ...<br/><br/>U Suter, R Bastos, H Hofstetter (1987) <i>Nucleic Acids Res</i> <i></i> <i></i> 15: 18 7295-7308<br/>