نتایج جستجو برای: sporadic amyotrophic lateral sclerosis sals

تعداد نتایج: 198646  

Journal: :Journal of molecular biology 2011
Elizabeth A Proctor Feng Ding Nikolay V Dokholyan

Aggregation of Cu,Zn superoxide dismutase (SOD1) is implicated in amyotrophic lateral sclerosis. Glutathionylation and phosphorylation of SOD1 is omnipresent in the human body, even in healthy individuals, and has been shown to increase SOD1 dimer dissociation, which is the first step on the pathway toward SOD1 aggregation. We found that post-translational modification of SOD1, especially gluta...

2012
Azusa Uchida Hiroki Sasaguri Nobuyuki Kimura Mio Tajiri Takuya Ohkubo Fumiko Ono Fumika Sakaue Kazuaki Kanai Takashi Hirai Tatsuhiko Sano Kazumoto Shibuya Masaki Kobayashi Mariko Yamamoto Shigefumi Yokota Takayuki Kubodera Masaki Tomori Kyohei Sakaki Mitsuhiro Enomoto Yukihiko Hirai Jiro Kumagai Yasuhiro Yasutomi Hideki Mochizuki Satoshi Kuwabara Toshiki Uchihara Hidehiro Mizusawa Takanori Yokota

Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by progressive motoneuron loss. Redistribution of transactive response deoxyribonucleic acid-binding protein 43 from the nucleus to the cytoplasm and the presence of cystatin C-positive Bunina bodies are considered pathological hallmarks of amyotrophic lateral sclerosis, but their significance has not been fully el...

Journal: :Brain : a journal of neurology 2012
Adriano Chiò Giuseppe Borghero Gabriella Restagno Gabriele Mora Carsten Drepper Bryan J Traynor Michael Sendtner Maura Brunetti Irene Ossola Andrea Calvo Maura Pugliatti Maria Alessandra Sotgiu Maria Rita Murru Maria Giovanna Marrosu Francesco Marrosu Kalliopi Marinou Jessica Mandrioli Patrizia Sola Claudia Caponnetto Gianluigi Mancardi Paola Mandich Vincenzo La Bella Rossella Spataro Amelia Conte Maria Rosaria Monsurrò Gioacchino Tedeschi Fabrizio Pisano Ilaria Bartolomei Fabrizio Salvi Giuseppe Lauria Pinter Isabella Simone Giancarlo Logroscino Antonio Gambardella Aldo Quattrone Christian Lunetta Paolo Volanti Marcella Zollino Silvana Penco Stefania Battistini Alan E Renton Elisa Majounie Yevgeniya Abramzon Francesca Luisa Conforti Fabio Giannini Massimo Corbo Mario Sabatelli

A large hexanucleotide (GGGGCC) repeat expansion in the first intron of C9ORF72, a gene located on chromosome 9p21, has been recently reported to be responsible for ~40% of familial amyotrophic lateral sclerosis cases of European ancestry. The aim of the current article was to describe the phenotype of amyotrophic lateral sclerosis cases carrying the expansion by providing a detailed clinical d...

Journal: :American journal of human genetics 2012
Nancy D Merner Simon L Girard Hélène Catoire Cynthia V Bourassa Véronique V Belzil Jean-Baptiste Rivière Pascale Hince Annie Levert Alexandre Dionne-Laporte Dan Spiegelman Anne Noreau Sabrina Diab Anna Szuto Hélène Fournier John Raelson Majid Belouchi Michel Panisset Patrick Cossette Nicolas Dupré Geneviève Bernard Sylvain Chouinard Patrick A Dion Guy A Rouleau

Essential tremor (ET) is a common neurodegenerative disorder that is characterized by a postural or motion tremor. Despite a strong genetic basis, a gene with rare pathogenic mutations that cause ET has not yet been reported. We used exome sequencing to implement a simple approach to control for misdiagnosis of ET, as well as phenocopies involving sporadic and senile ET cases. We studied a larg...

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