Patrick Weydt's Publications List

Towards a European Registry and Biorepository for Patients with Spinal and Bulbar Muscular Atrophy.

2016-02-03T12:03:53Z

Pathomechanisms of spinal and bulbar muscular atrophy (SBMA) have been extensively investigated and are partially understood, but no effective treatment is currently available for this disabling disorder. Its rarity, the slow disease progression, and lack of sensitive-to-change outcome measures render design and conduction of clinical trials a challenging task. Therefore, it is fundamental to stre...

Davide Pareyson, Pietro Fratta, Pierre-François Pradat, Gianni Sorarù, Josef Finsterer, John Vissing, Manu E Jokela, Bjarne Udd, Albert C Ludolph, Anna Sagnelli, Patrick Weydt (2016) Journal of molecular neuroscience : MN :

Neurofilament levels as biomarkers in asymptomatic and symptomatic familial amyotrophic lateral sclerosis.

2016-02-03T12:03:53Z

Neurofilaments are elevated in the cerebrospinal fluid (CSF) and serum of amyotrophic lateral sclerosis (ALS) patients. However, timing of this increase is unknown. To characterize the premanifest disease phase, we performed a cross-sectional study on asymptomatic (n = 12) and symptomatic (n = 64) ALS mutation carriers and family controls (n = 19). Neurofilaments NF-L (neurofilament-li...

Patrick Weydt, Patrick Oeckl, André Huss, Kathrin Müller, Alexander E Volk, Jens Kuhle, Antje Knehr, Peter M Andersen, Johannes Prudlo, Petra Steinacker, Jochen H Weishaupt, Albert C Ludolph, Markus Otto (2016) Annals of neurology 79: 1 152-158

Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients.

2016-02-03T12:03:53Z

Biomarkers for the diagnosis of motoneuron diseases (MND) are urgently needed to improve the diagnostic pathway, patient stratification and monitoring. The aim of this study was to validate candidate markers for MND in cerebrospinal fluid (CSF) and specify cut-offs based on large patient cohorts by especially considering patients who were seen under the initial differential diagnosis (MND mimics).

Petra Steinacker, Emily Feneberg, Jochen Weishaupt, Johannes Brettschneider, Hayrettin Tumani, Peter M Andersen, Christine A F von Arnim, Sarah Böhm, Jan Kassubek, Christian Kubisch, Dorothée Lulé, Hans-Peter Müller, Rainer Muche, Elmar Pinkhardt, Patrick Oeckl, Angela Rosenbohm, Sarah Anderl-Straub, Alexander E Volk, Patrick Weydt, Albert C Ludolph, Markus Otto (2016) Journal of neurology, neurosurgery, and psychiatry 87: 1 12-20

Comparative biochemical characterization of the monoacylglycerol lipase inhibitor KML29 in brain, spinal cord, liver, spleen, fat and muscle tissue.

2016-02-03T12:03:53Z

Monoacylglycerol lipase (MAGL) is part of the endocannabinoid and the prostaglandin signaling system. MAGL degrades the endocannabinoid 2-arachidonoylglycerol (2-AG) into glycerol and arachidonic acid. MAGL-induced arachidonic acid is the primary source for prostaglandin synthesis in the brain. 2-AG mainly induces neuroprotective and anti-inflammatory effects, whereas prostaglandins are related to...

Noemi Pasquarelli, Christoph Porazik, Johannes Hanselmann, Patrick Weydt, Boris Ferger, Anke Witting (2015) Neuropharmacology 91: 148-156

Clinical Trials in Spinal and Bulbar Muscular Atrophy-Past, Present, and Future.

2016-02-03T12:03:53Z

Spinal and Bulbar Muscular Atrophy (SBMA), also known as Kennedy's disease, is a rare adult-onset lower motor neuron disorder with a classic X-linked inheritance pattern. It is caused by the abnormal expansion of the CAG-repeat tract in the androgen receptor gene. Despite important progress in the understanding of the molecular pathogenesis and the availability of a broad set of model organisms, s...

Patrick Weydt, Anna Sagnelli, Angela Rosenbohm, Pietro Fratta, Pierre-François Pradat, Albert C Ludolph, Davide Pareyson (2015) Journal of molecular neuroscience : MN :

Screening for CHCHD10 mutations in a large cohort of sporadic ALS patients: no evidence for pathogenicity of the p.P34S variant.

2016-02-03T12:03:53Z

Nicolai Marroquin, Sebastian Stranz, Kathrin Müller, Thomas Wieland, Wolfgang P Ruf, Sarah J Brockmann, Karin M Danzer, Guntram Borck, Annemarie Hübers, Patrick Weydt, Thomas Meitinger, Tim-Matthias Strom, Angela Rosenbohm, Albert C Ludolph, Jochen H Weishaupt (2015) Brain : a journal of neurology :

Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization.

2016-02-03T12:03:53Z

Aggregation of α-synuclein (α-syn) and α-syn cytotoxicity are hallmarks of sporadic and familial Parkinson disease (PD), with accumulating evidence that prefibrillar oligomers and protofibrils are the pathogenic species in PD and related synucleinopathies. Peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α), a key regulator of mitochondrial biogenesis and cellular energy met...

Judith Eschbach, Björn von Einem, Kathrin Müller, Hanna Bayer, Annika Scheffold, Bradley E Morrison, K Lenhard Rudolph, Dietmar R Thal, Anke Witting, Patrick Weydt, Markus Otto, Michael Fauler, Birgit Liss, Pamela J McLean, Albert R La Spada, Albert C Ludolph, Jochen H Weishaupt, Karin M Danzer (2015) Annals of neurology 77: 1 15-32

Serum microRNAs in sporadic amyotrophic lateral sclerosis.

2016-02-03T12:03:53Z

MicroRNAs (miRNAs) are post-transcriptional regulators of gene expression and specific mircoRNA "fingerprints" are thought to contribute to and/or reflect certain disease conditions. Recently, we identified surprisingly homogeneous signatures of circulating miRNAs in the serum of familial amyotrophic lateral sclerosis (ALS) patients, which were already present in presymptomatic carriers of ALS gen...

Axel Freischmidt, Kathrin Müller, Lisa Zondler, Patrick Weydt, Benjamin Mayer, Christine A F von Arnim, Annemarie Hübers, Johannes Dorst, Markus Otto, Karlheinz Holzmann, Albert C Ludolph, Karin M Danzer, Jochen H Weishaupt (2015) Neurobiology of aging 36: 9 2660.e15-2660.e20

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia.

2016-02-03T12:03:53Z

Amyotrophic lateral sclerosis (ALS) is a genetically heterogeneous neurodegenerative syndrome hallmarked by adult-onset loss of motor neurons. We performed exome sequencing of 252 familial ALS (fALS) and 827 control individuals. Gene-based rare variant analysis identified an exome-wide significant enrichment of eight loss-of-function (LoF) mutations in TBK1 (encoding TANK-binding kinase 1) in 13 f...

Axel Freischmidt, Thomas Wieland, Benjamin Richter, Wolfgang Ruf, Veronique Schaeffer, Kathrin Müller, Nicolai Marroquin, Frida Nordin, Annemarie Hübers, Patrick Weydt, Susana Pinto, Rayomond Press, Stéphanie Millecamps, Nicolas Molko, Emilien Bernard, Claude Desnuelle, Marie-Hélène Soriani, Johannes Dorst, Elisabeth Graf, Ulrika Nordström, Marisa S Feiler, Stefan Putz, Tobias M Boeckers, Thomas Meyer, Andrea S Winkler, Juliane Winkelman, Mamede de Carvalho, Dietmar R Thal, Markus Otto, Thomas Brännström, Alexander E Volk, Petri Kursula, Karin M Danzer, Peter Lichtner, Ivan Dikic, Thomas Meitinger, Albert C Ludolph, Tim M Strom, Peter M Andersen, Jochen H Weishaupt (2015) Nature neuroscience 18: 5 631-636

Development, implementation, and evaluation of a movie-based curriculum to teach psychopathology.

2016-02-03T12:04:39Z

Because medical students' attitudes toward psychiatry are often fostered by media, we provided an elective movie-based seminar to teach psychopathology.

Heiko Graf, Birgit Abler, Patrick Weydt, Thomas Kammer, Paul L Plener (2014) Teaching and learning in medicine 26: 1 86-89

A single nucleotide polymorphism in the coding region of PGC-1α is a male-specific modifier of Huntington disease age-at-onset in a large European cohort.

2016-02-03T12:04:39Z

Genetic modifiers are important clues for the identification of therapeutic targets in neurodegenerative diseases. Huntington disease (HD) is one of the most common autosomal dominant inherited neurodegenerative diseases. The clinical symptoms include motor abnormalities, cognitive decline and behavioral disturbances. Symptom onset is typically between 40 and 50 years of age, but can vary by sever...

Patrick Weydt, Selma M Soyal, G Bernhard Landwehrmeyer, Wolfgang Patsch, (2014) BMC neurology 14:

Incidence and geographical variation of amyotrophic lateral sclerosis (ALS) in Southern Germany--completeness of the ALS registry Swabia.

2016-02-03T12:04:39Z

Objective of this paper was to investigate the incidence, potential geographical clusters and the completeness of the amyotrophic lateral sclerosis (ALS) registry in Southern Germany (Swabia). Age-standardized incidence rates (ASR) and ratios (SIR) as well as 95% confidence intervals (CI) were estimated at county level. Capture-recapture (CARE) procedures were applied taking data source dependency...

Hatice Uenal, Angela Rosenbohm, Johannes Kufeldt, Patrick Weydt, Katharina Goder, Albert Ludolph, Dietrich Rothenbacher, Gabriele Nagel, (2014) PloS one 9: 4

Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers.

2016-02-03T12:04:39Z

Knowledge about the nature of pathomolecular alterations preceding onset of symptoms in amyotrophic lateral sclerosis is largely lacking. It could not only pave the way for the discovery of valuable therapeutic targets but might also govern future concepts of pre-manifest disease modifying treatments. MicroRNAs are central regulators of transcriptome plasticity and participate in pathogenic cascad...

Axel Freischmidt, Kathrin Müller, Lisa Zondler, Patrick Weydt, Alexander E Volk, Anže Lošdorfer Božič, Michael Walter, Michael Bonin, Benjamin Mayer, Christine A F von Arnim, Markus Otto, Christoph Dieterich, Karlheinz Holzmann, Peter M Andersen, Albert C Ludolph, Karin M Danzer, Jochen H Weishaupt (2014) Brain : a journal of neurology 137: Pt 11 2938-2950

Prodromal Huntington disease as a model for functional compensation of early neurodegeneration.

2016-02-03T12:03:53Z

Functional compensation demonstrated as mechanism to offset neuronal loss in early Alzheimer disease may also occur in other adult-onset neurodegenerative diseases, particularly Huntington disease (HD) with its genetic determination and gradual changes in structural integrity. In HD, neurodegeneration typically initiates in the dorsal striatum, successively affecting ventral striatal areas. Invest...

Kathrin Malejko, Patrick Weydt, Sigurd D Süßmuth, Georg Grön, Bernhard G Landwehrmeyer, Birgit Abler (2014) PloS one 9: 12

Can lesions to the motor cortex induce amyotrophic lateral sclerosis?

2016-02-03T12:04:39Z

A recent staging effort for amyotrophic lateral sclerosis (ALS) has demonstrated that the TDP-43 neuropathology may initiate focally in the motor cortex in the majority of patients. We searched our data bank for patients with lesions of the motor cortex which preceded disease onset. We performed a search of our patient- and MRI-data bank and screened 1,835 patients with amyotrophic lateral scleros...

Angela Rosenbohm, Jan Kassubek, Patrick Weydt, Nicolai Marroquin, Alexander E Volk, Christian Kubisch, Hans-Jürgen Huppertz, Markus Weber, Peter M Andersen, Jochen H Weishaupt, Albert C Ludolph, (2014) Journal of neurology 261: 2 283-290

Two-point magnitude MRI for rapid mapping of brown adipose tissue and its application to the R6/2 mouse model of Huntington disease.

2016-02-03T12:04:39Z

The recent discovery of active brown fat in human adults has led to renewed interest in the role of this key metabolic tissue. This is particularly true for neurodegenerative conditions like Huntington disease (HD), an adult-onset heritable disorder with a prominent energy deficit phenotype. Current methods for imaging brown adipose tissue (BAT) are in limited use because they are equipment-wise d...

Katrin S Lindenberg, Patrick Weydt, Hans-Peter Müller, Axel Bornstedt, Albert C Ludolph, G Bernhard Landwehrmeyer, Wolfgang Rottbauer, Jan Kassubek, Volker Rasche (2014) PloS one 9: 8

Neuropathology of partial PGC-1α deficiency recapitulates features of mitochondrial encephalopathies but not of neurodegenerative diseases.

2016-02-03T12:04:39Z

Deficient peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) function is one component of mitochondrial dysfunction in neurodegenerative diseases. Current molecular classification of such diseases is based on the predominant protein accumulating as intra- or extracellular aggregates. Experimental evidence suggests that mitochondrial dysfunction and impaired protein processing ...

Levente Szalardy, Denes Zadori, Imola Plangar, Laszlo Vecsei, Patrick Weydt, Albert C Ludolph, Peter Klivenyi, Gabor G Kovacs (2013) Neuro-degenerative diseases 12: 4 177-188

Full-length PGC-1α salvages the phenotype of a mouse model of human neuropathy through mitochondrial proliferation.

2016-02-03T12:04:39Z

Increased mitochondrial mass, commonly termed mitochondrial proliferation, is frequently observed in many human diseases directly or indirectly involving mitochondrial dysfunction. Mitochondrial proliferation is thought to counterbalance a compromised energy metabolism, yet it might also be detrimental through alterations of mitochondrial regulatory functions such as apoptosis, calcium metabolism ...

Krisztina Rona-Voros, Judith Eschbach, Aurélia Vernay, Diana Wiesner, Birgit Schwalenstocker, Pauline Geniquet, Bénédicte Mousson De Camaret, Andoni Echaniz-Laguna, Jean-Philippe Loeffler, Albert C Ludolph, Patrick Weydt, Luc Dupuis (2013) Human molecular genetics 22: 25 5096-5106

PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis.

2016-02-03T12:04:39Z

Amyotrophic lateral sclerosis (ALS) is a devastating, adult-onset neurodegenerative disorder of the upper and lower motor systems. It leads to paresis, muscle wasting and inevitably to death, typically within 3-5 years. However, disease onset and survival vary considerably ranging in extreme cases from a few months to several decades. The genetic and environmental factors underlying this variabili...

Judith Eschbach, Birgit Schwalenstöcker, Selma M Soyal, Hanna Bayer, Diana Wiesner, Chizuru Akimoto, Ann-Charloth Nilsson, Anna Birve, Thomas Meyer, Luc Dupuis, Karin M Danzer, Peter M Andersen, Anke Witting, Albert C Ludolph, Wolfgang Patsch, Patrick Weydt (2013) Human molecular genetics 22: 17 3477-3484

Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age.

2016-02-03T12:04:39Z

Mutations in the DYNC1H1 gene encoding for dynein heavy chain cause two closely related human motor neuropathies, dominant spinal muscular atrophy with lower extremity predominance (SMA-LED) and axonal Charcot-Marie-Tooth (CMT) disease, and lead to sensory neuropathy and striatal atrophy in mutant mice. Dynein is the molecular motor carrying mitochondria retrogradely on microtubules, yet the conse...

Judith Eschbach, Jérôme Sinniger, Jamal Bouitbir, Anissa Fergani, Anna-Isabel Schlagowski, Joffrey Zoll, Bernard Geny, Frédérique René, Yves Larmet, Vincent Marion, Robert H Baloh, Matthew B Harms, Michael E Shy, Nadia Messadeq, Patrick Weydt, Jean-Philippe Loeffler, Albert C Ludolph, Luc Dupuis (2013) Neurobiology of disease 58: 220-230

Progranulin bridges energy homeostasis and fronto-temporal dementia.

2012-03-20T21:20:22Z

Luc Dupuis, Asa Petersen, Patrick Weydt (2012) Cell Metab 15: 3 269-270

A greatly extended PPARGC1A genomic locus encodes several new brain-specific isoforms and influences Huntington disease age of onset.

2012-10-20T12:20:04Z

PGC-1α has been implicated in the pathogenesis of neurodegenerative disorders. Several single-nucleotide polymorphisms (SNPs) located in two separate haplotype blocks of PPARGC1A have shown associations with Huntington's disease (HD) and Parkinson's disease, but causative SNPs have not been identified. One SNP (rs7665116) was located in a highly conserved 233 bp region of intron 2. To determine w...

Selma M Soyal, Thomas K Felder, Simon Auer, Penelope Hahne, Hannes Oberkofler, Anke Witting, Markus Paulmichl, G Bernhard Landwehrmeyer, Patrick Weydt, Wolfgang Patsch (2012) Hum Mol Genet 21: 15 3461-3473

Hypogonadism and gynecomastia with duloxetine.

2012-03-20T21:18:49Z

P Weydt, C J Schönfeldt-Lecuona, M Gahr, B J Connemann (2011) Pharmacopsychiatry 44: 2

The Role of PGC-1alpha in the Pathogenesis of Neurodegenerative Disorders.

2012-03-20T21:20:22Z

Mitochondrial dysfunction is a common hallmark of ageing-related diseases involving neurodegeneration. Huntington's disease (HD) is one of the most common monogenetic forms of neurodegenerative disorders and shares many salient features with the major sporadic disease of neurodegeneration, such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD) and Parkinson's disease (PD). Recent ev...

Krisztina Róna-Vörös, Patrick Weydt (2010) Curr Drug Targets :

Mild cognitive impairment in prediagnosed Huntington disease.

2012-03-20T21:20:22Z

Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current study examined the rates of subtle cognitive dysfunct...

K Duff, J Paulsen, J Mills, L J Beglinger, D J Moser, M M Smith, D Langbehn, J Stout, S Queller, D L Harrington (2010) Neurology 75: 6 500-507

The gene coding for PGC-1alpha modifies age at onset in Huntington's Disease.

2012-03-20T21:20:22Z

ABSTRACT: Huntington's disease (HD) is one of the most common autosomal dominant inherited, neurodegenerative disorders. It is characterized by progressive motor, emotional and cognitive dysfunction. In addition metabolic abnormalities such as wasting and altered energy expenditure are increasingly recognized as clinical hallmarks of the disease. HD is caused by an unstable CAG repeat expansion in...

Patrick Weydt, Selma M Soyal, Cinzia Gellera, Stefano Didonato, Claus Weidinger, Hannes Oberkofler, G Bernhard Landwehrmeyer, Wolfgang Patsch (2009) Mol Neurodegener 4: 1

Targeting protein aggregation in neurodegeneration--lessons from polyglutamine disorders.

2008-03-18T11:24:54Z

Polyglutamine diseases, such as Huntington's disease, are among the most common inherited neurodegenerative disorders. They share salient clinical and pathological features with major sporadic neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease and amyotropic lateral sclerosis. Over the last decade, protein aggregation has emerged as a common pathological hallmark in neuro...

Patrick Weydt, Albert R La Spada (2006) Expert Opin Ther Targets 10: 4 505-513

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.

2008-03-18T11:24:54Z

Huntington's disease (HD) is a fatal, dominantly inherited disorder caused by polyglutamine repeat expansion in the huntingtin (htt) gene. Here, we observe that HD mice develop hypothermia associated with impaired activation of brown adipose tissue (BAT). Although sympathetic stimulation of PPARgamma coactivator 1alpha (PGC-1alpha) was intact in BAT of HD mice, uncoupling protein 1 (UCP-1) inducti...

Patrick Weydt, Victor V Pineda, Anne E Torrence, Randell T Libby, Terrence F Satterfield, Eduardo R Lazarowski, Merle L Gilbert, Gregory J Morton, Theodor K Bammler, Andrew D Strand, Libin Cui, Richard P Beyer, Courtney N Easley, Annette C Smith, Dimitri Krainc, Serge Luquet, Ian R Sweet, Michael W Schwartz, Albert R La Spada (2006) Cell Metab 4: 5 349-362

Modafinil to treat fatigue in amyotrophic lateral sclerosis: an open label pilot study.

2008-03-18T11:24:54Z

An open label trial of modafinil was conducted to determine whether it would be tolerated and effective in treating fatigue for people with amyotrophic lateral sclerosis (ALS). Fifteen patients with ALS were treated for two weeks with either 200 mg or 400 mg of modafinil. Reported side effects of the medication were mild and included diarrhea, headache, nervousness, and insomnia. Side effects did ...

Gregory T Carter, Michael D Weiss, Jau-Shin Lou, Mark P Jensen, R Ted Abresch, Tara K Martin, Thomas W Hecht, Jay J Han, Patrick Weydt, George H Kraft (2005) Am J Hosp Palliat Care 22: 1 55-59

Cannabinol delays symptom onset in SOD1 (G93A) transgenic mice without affecting survival.

2008-03-18T11:24:54Z

Therapeutic options for amyotrophic lateral sclerosis (ALS), the most common adult-onset motor neuron disorder, remain limited. Emerging evidence from clinical studies and transgenic mouse models of ALS suggests that cannabinoids, the bioactive ingredients of marijuana (Cannabis sativa) might have some therapeutic benefit in this disease. However, Delta(9)-tetrahydrocannabinol (Delta(9)-THC), the ...

Patrick Weydt, Soyon Hong, Anke Witting, Thomas Möller, Nephi Stella, Michel Kliot (2005) Amyotroph Lateral Scler Other Motor Neuron Disord 6: 3 182-184

Neuroinflammation in the pathogenesis of amyotrophic lateral sclerosis.

2008-03-18T11:24:54Z

Amyotrophic lateral sclerosis is a devastating motor neuron disorder. Traditionally regarded as a 'neuron only' disease, recent evidence suggested that other cells contribute critically to the pathogenesis. This review provides a short synopsis of the role neuroinflammation and microglial cells play in the disease and its animal models. A better understanding of neuroinflammation in motor neuron d...

Patrick Weydt, Thomas Möller (2005) Neuroreport 16: 6 527-531

Targeting toxic proteins for turnover.

2008-03-18T11:24:54Z

Albert R La Spada, Patrick Weydt (2005) Nat Med 11: 10 1052-1053

Skeletal muscle in amyotrophic lateral sclerosis: emerging concepts and therapeutic implications.

2008-03-18T11:24:54Z

Simone Abmayr, Patrick Weydt (2005) Phys Med Rehabil Clin N Am 16: 4 1091-7, xi-xii

The role of microglial cells in amyotrophic lateral sclerosis.

2008-03-18T11:24:54Z

Patrick Weydt, Thomas Möller (2005) Phys Med Rehabil Clin N Am 16: 4 1081-90, xi

Survey of cannabis use in patients with amyotrophic lateral sclerosis.

2008-03-18T11:24:54Z

Cannabis (marijuana) has been proposed as treatment for a widening spectrum of medical conditions and has many properties that may be applicable to the management of amyotrophic lateral sclerosis (ALS). This study is the first, anonymous survey of persons with ALS regarding the use of cannabis. There were 131 respondents, 13 of whom reported using cannabis in the last 12 months. Although the small...

Dagmar Amtmann, Patrick Weydt, Kurt L Johnson, Mark P Jensen, Gregory T Carter (2004) Am J Hosp Palliat Care 21: 2 95-104

Medicinal cannabis: rational guidelines for dosing.

2008-03-18T11:24:54Z

The medicinal value of cannabis (marijuana) is well documented in the medical literature. Cannabinoids, the active ingredients in cannabis, have many distinct pharmacological properties. These include analgesic, anti-emetic, anti-oxidative, neuroprotective and anti-inflammatory activity, as well as modulation of glial cells and tumor growth regulation. Concurrent with all these advances in the und...

Gregory T Carter, Patrick Weydt, Muraco Kyashna-Tocha, Donald I Abrams (2004) IDrugs 7: 5 464-470

Increased cytotoxic potential of microglia from ALS-transgenic mice.

2008-03-18T11:24:54Z

Amyotrophic lateral sclerosis is a fatal, adult-onset motor neuron disease. A subset of cases is caused by mutations of superoxide dismutase 1 (SOD1) gene. The mechanisms how the mutations in this ubiquitous enzyme mediate the highly selective motor neuron degeneration, however, remain poorly understood. Recent results from transgenic animal models suggest a "non-cell autonomous" mechanism; i.e., ...

Patrick Weydt, Eric C Yuen, Bruce R Ransom, Thomas Möller (2004) Glia 48: 2 179-182

Current pharmacological management of amyotrophic [corrected] lateral sclerosis and a role for rational polypharmacy.

2008-03-18T11:24:54Z

Amyotrophic [corrected] lateral sclerosis (ALS) is a progressive degenerative condition of motor neurons that is ultimately fatal. Even though scientific discovery over the past few decades has led to a greater understanding of the pathogenic mechanisms of ALS, effective pharmacotherapy intended to slow, arrest or reverse the disease progression remains difficult to obtain. Riluzole, a drug that h...

Michael D Weiss, Patrick Weydt, Gregory T Carter (2004) Expert Opin Pharmacother 5: 4 735-746

Inflammatory mediators and growth factors in the spinal cord of G93A SOD1 rats.

2008-03-18T11:24:54Z

Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease. One mechanism involved in ALS pathology is neuroinflammation. Neuroinflammation is mediated by soluble pro-inflammatory molecules such as cytokines, prostaglandins and nitric oxide. Studies on transgenic mice demonstrated the expression of pro-inflammatory mediators in early stages of murine ALS. Recently a transgenic rat m...

Yiheng Xie, Patrick Weydt, David S Howland, Michel Kliot, Thomas Möller (2004) Neuroreport 15: 16 2513-2516

Endocannabinoids accumulate in spinal cord of SOD1 G93A transgenic mice.

2008-03-18T11:24:54Z

Approximately 2% of amyotrophic lateral sclerosis (ALS) cases are caused by mutations in the super oxide dismutase 1 (SOD1) gene and transgenic mice for these mutations recapitulate many features of this devastating neurodegenerative disease. Here we show that the amount of anandamide (AEA) and 2-arachidonoylglycerol (2-AG), two endocannabinoids that have neuroprotective properties, increase in sp...

Anke Witting, Patrick Weydt, Soyon Hong, Michel Kliot, Thomas Moller, Nephi Stella (2004) J Neurochem 89: 6 1555-1557

Assessing disease onset and progression in the SOD1 mouse model of ALS.

2008-03-18T11:24:54Z

SOD1 transgenic mice are the most widely used animal model of amyotrophic lateral sclerosis (ALS). In addition to providing valuable insights into the pathogenesis of ALS, these animals are used intensively in many laboratories as an in vivo model for investigating novel therapeutic interventions towards this devastating motorneuron disease. Such pre-clinical studies require objective and reliable...

Patrick Weydt, So Yon Hong, Michel Kliot, Thomas Möller (2003) Neuroreport 14: 7 1051-1054

Electrodiagnostic evaluation of hereditary motor and sensory neuropathies.

2008-03-18T11:24:54Z

Electrodiagnosis can classify hereditary motor and sensory neuropathies (HMSN) into two basic types: primarily demyelinating with secondary axonal loss and primarily axonal. For the most part, the various forms of HMSN show uniform symmetric nerve conduction slowing, in contrast to acquired neuropathies, which may be multifocal with nonuniform conduction velocity slowing and temporal dispersion. N...

Gregory T Carter, John D England, Thomas W Hecht, Jay J Han, Patrick Weydt, Phillip F Chance (2003) Phys Med Rehabil Clin N Am 14: 2 347-63, ix-x

Drug therapy for amyotrophic lateral sclerosis: Where are we now?

2008-03-18T11:24:54Z

In the 60 years since Lou Gehrig died from amyotrophic lateral sclerosis (ALS) there have been numerous advances in our understanding of this disease. However, scant progress has been made regarding disease-altering treatments. Today most physicians still recommend vitamin E, which is the treatment Gehrig himself received. In this paper we will review what is currently known about the pathophysiol...

Gregory T Carter, Lisa S Krivickas, Patrick Weydt, Michael D Weiss, Robert G Miller (2003) IDrugs 6: 2 147-153

Cannabis: old medicine with new promise for neurological disorders.

2008-03-18T11:24:54Z

Marijuana is a complex substance containing over 60 different forms of cannabinoids, the active ingredients. Cannabinoids are now known to have the capacity for neuromodulation, via direct, receptor-based mechanisms at numerous levels within the nervous system. These have therapeutic properties that may be applicable to the treatment of neurological disorders; including anti-oxidative, neuroprotec...

Gregory T Carter, Patrick Weydt (2002) Curr Opin Investig Drugs 3: 3 437-440

Neuro-inflammation as a therapeutic target in amyotrophic lateral sclerosis.

2008-03-18T11:24:54Z

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neuromuscular disease that destroys both upper and lower motor neurons, resulting in spasticity, diffuse muscular atrophy, weakness, and ultimately death from respiratory failure. It is presumed that in the vast majority of cases, ALS is acquired and occurs sporadically, although the exact etiology is unknown. Recent, emerging evidence s...

Patrick Weydt, Michael D Weiss, Thomas Möller, Gregory T Carter (2002) Curr Opin Investig Drugs 3: 12 1720-1724

Biomedical centre memorial to victims of Nazi research.

2008-03-18T11:24:54Z

P Weydt (2000) Nature 403: 6772

Frustration grows over EU grant application procedures.

2008-03-18T11:24:54Z

A Abbott, P Weydt (2000) Nature 404: 6779

German research agency stifles creativity.

2008-03-18T11:24:54Z

Q Schiermeier, P Weydt (2000) Nature 404: 6775

Activation of metabotropic glutamate receptors delays apoptosis of chick embryonic motor neurons in vitro.

2008-03-18T11:24:54Z

Glutamatergic excitotoxicity has been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, activation of metabotropic glutamate receptors (mGluRs) is neuroprotective in several paradigms. We therefore tested the effect of selective mGluR agonists on cultured chick embryonic motor neurons. Activation of group I mGluRs with (s)-3,5-dihydroxyphenylglycine (DHPG) and group I...

J M Anneser, S Horstmann, P Weydt, G D Borasio (1998) Neuroreport 9: 9 2039-2043

Action potential-generating cells in human glioblastomas.

2008-03-18T11:24:54Z

We studied the electrophysiological properties of cells from human glioblastomas obtained after surgery. The membrane currents were compared in cells of acute tissue slices and primary cultures using the whole cell mode of the patch-clamp technique. Very strikingly, in about a third of the tumor cells in situ and in vitro, depolarizing voltage steps elicited large, tetrodotoxin-sensitive inward cu...

C Labrakakis, S Patt, P Weydt, J Cervós-Navarro, R Meyer, H Kettenmann (1997) J Neuropathol Exp Neurol 56: 3 243-254

Neuroligand-triggered calcium signalling in cultured human glioma cells.

2008-03-18T11:24:54Z

Cells from primary cultures of four glioblastomas (GB), three low-grade astrocytomas (A), and four low-grade oligodendrogliomas (O) were tested for the presence of neuroligand receptors linked to Ca2+ signalling by calcium imaging. Cells of days 3 to 21 in culture were incubated with 5 microM fluo-3-acetomethylester in a bath solution and stimulated with 0.1 mM ATP, 0.01 mM angiotensin II, bradyki...

P Weydt, T Möller, C Labrakakis, S Patt, H Kettenmann (1997) Neurosci Lett 228: 2 91-94

Human central neurocytoma cells show neuronal physiological properties in vitro.

2008-03-18T11:24:54Z

Central neurocytoma is a rare brain tumor composed of small round synaptophysin-positive cells, suggesting a neuronal origin of these tumor cells. Glial properties are inferred, however, from the observation that the tumor cells exhibit a strong morphological similarity to oligodendroglioma cells and show an astrocytic differentiation in vitro. To test for neuronal or glial physiological propertie...

S Patt, H Schmidt, C Labrakakis, P Weydt, M Fritsch, J Cervós-Navarro, H Kettenmann (1996) Acta Neuropathol 91: 2 209-214

Neuron-like physiological properties of cells from human oligodendroglial tumors.

2008-03-18T11:24:54Z

One of the most common symptoms of patients with oligodendrogliomas is the high frequency of epileptic seizures. We thus studied the physiological properties of cells in six human oligodendrogliomas and two oligoastrocytomas obtained from surgical material. The majority of tumor cells in living brain slices can generate action potentials as recorded with the patch-clamp technique indicating that t...

S Patt, C Labrakakis, M Bernstein, P Weydt, J Cervós-Navarro, G Nisch, H Kettenmann (1996) Neuroscience 71: 2 601-611