نتایج جستجو برای: als gene

تعداد نتایج: 1166211  

Journal: :Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 2006

2013
Motoko Kawaguchi-Niida Tomoko Yamamoto Yoichiro Kato Yuri Inose Noriyuki Shibata

BACKGROUND Emerging evidence suggests that innate immunity and increased oxidative stress contribute to pathomechanisms in amyotrophic lateral sclerosis (ALS). The aim of the present study was to verify the involvement of monocyte chemoattractant protein-1 (MCP-1) and its specific CC chemokine receptor 2 (CCR2) in the disease progression of ALS. We here demonstrate the expression state of MCP-1...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2012
Jumpei Sasabe Yurika Miyoshi Masataka Suzuki Masashi Mita Ryuichi Konno Masaaki Matsuoka Kenji Hamase Sadakazu Aiso

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder involving an extensive loss of motoneurons. Aberrant excitability of motoneurons has been implicated in the pathogenesis of selective motoneuronal death in ALS. D-serine, an endogenous coagonist of N-methyl-D-aspartate receptors, exacerbates motoneuronal death and is increased both in patients with sporadic/familial ALS a...

2014
Nadhim Bayatti Johnathan Cooper-Knock Joanna J. Bury Matthew Wyles Paul R. Heath Janine Kirby Pamela J. Shaw

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes death within a mean of 2-3 years from symptom onset. There is no diagnostic test and the delay from symptom onset to diagnosis averages 12 months. The identification of prognostic and diagnostic biomarkers in ALS would facilitate earlier diagnosis and faster monitoring of treatments. Gene expression profiling (GEP) c...

Journal: :Neurobiology of aging 2012
Ewout J N Groen Wouter van Rheenen Max Koppers Perry T C van Doormaal Lotte Vlam Frank P Diekstra Dennis Dooijes R Jeroen Pasterkamp Leonard H van den Berg Jan H Veldink

Recently, repeat expansions in several genes have been shown to cause or be associated with amyotrophic lateral sclerosis (ALS). It has been demonstrated that an intronic hexanucleotide repeat expansion in C9ORF72 is a major cause of both familial (approximately 40%) and sporadic (approximately 5%) ALS, as well as frontotemporal dementia (FTD). In addition, a CAG-repeat expansion in exon 1 of A...

Journal: :Neuron 2011
Mariely DeJesus-Hernandez Ian R. Mackenzie Bradley F. Boeve Adam L. Boxer Matt Baker Nicola J. Rutherford Alexandra M. Nicholson NiCole A. Finch Heather Flynn Jennifer Adamson Naomi Kouri Aleksandra Wojtas Pheth Sengdy Ging-Yuek R. Hsiung Anna Karydas William W. Seeley Keith A. Josephs Giovanni Coppola Daniel H. Geschwind Zbigniew K. Wszolek Howard Feldman David S. Knopman Ronald C. Petersen Bruce L. Miller Dennis W. Dickson Kevin B. Boylan Neill R. Graff-Radford Rosa Rademakers

Several families have been reported with autosomal-dominant frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), genetically linked to chromosome 9p21. Here, we report an expansion of a noncoding GGGGCC hexanucleotide repeat in the gene C9ORF72 that is strongly associated with disease in a large FTD/ALS kindred, previously reported to be conclusively linked to chromosome 9p. T...

2014
Jeffrey S. Deitch Guillermo M. Alexander Andrew Bensinger Steven Yang Juliann T. Jiang Terry D. Heiman-Patterson

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the motor neuron. While most cases of ALS are sporadic, 10% are familial (FALS) with 20% of FALS caused by a mutation in the gene that codes for the enzyme Cu/Zn superoxide dismutase (SOD1). There is variability in sporadic ALS as well as FALS where even within the same family some siblings with the same mutation ...

Journal: :Blood 2017
Taxiarchis V Kourelis Surendra Dasari Jason D Theis Marina Ramirez-Alvarado Paul J Kurtin Morie A Gertz Steven R Zeldenrust Roman M Zenka Ahmet Dogan Angela Dispenzieri

The goal of this study was to investigate the frequency of use of light-chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad, and solid organ tissue samples typed by liquid ...

Journal: :Rinsho shinkeigaku = Clinical neurology 2011
Naoki Atsuta Ryoichi Nakamura Hazuki Watanabe Hirohisa Watanabe Mizuki Ito Jo Senda Fumiaki Tanaka Gen Sobue

To investigate the longitudinal course of Japanese patients with Amyotrophic Lateral Sclerosis (ALS), we constructed a multicenter registration and follow-up system called Japanese Consortium for Amyotrophic Lateral Sclerosis research (JaCALS). Genomic DNA samples of ALS patients were stored and linked to the clinical information. We designed a telephone survey system using a clinical research ...

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