Misfolded CuZnSOD and amyotrophic lateral sclerosis
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چکیده
منابع مشابه
Misfolded CuZnSOD and amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of motor neurons. The inherited form of the disease, familial ALS, represents 5-10% of the total cases, and the best documented of these are due to lesions in SOD1, the gene encoding copper-zinc superoxide dismutase (CuZnSOD). The mechanism by which mutations in SOD1 cause familial ALS is currently unknown. Two hypotheses...
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inherited loci map to chromosomes 2q and 15q whereas one dominant juvenile-onset locus maps to chromo-However, the genes responsible for these familial ALS-FTD and juvenile ALS cases have not yet been Amyotrophic lateral sclerosis (ALS) is one of the most identified. common adult-onset neurogenerative diseases, having In a small number of sporadic ALS patients (%1ف of a prevalence of 5ف per...
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Touch MEdical MEdia 53 Fasciculation, which is observed in various peripheral motor neuron disorders, is most frequently found in nonprogressive benign fasciculation syndrome (BFS). Patients with BFS never exhibit muscle atrophy or pathologic weakness and not all such patients visit hospitals; therefore, the prevalence and characteristics of BFS still need to be established. However, among the ...
متن کامل[Amyotrophic lateral sclerosis].
Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the motor system characterized by signs and symptoms of upper and lower motor dysfunction. This results in the presence of focal amyotrophies and pareses affecting voluntary muscles. Patients die after a few years, in most cases by respiratory failure. ALS is the most frequent motor neuron disease; however, its etiolo...
متن کاملAmyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting 20,000 to 30,000 people in the United States. The mainstay of care of patients affected by this disease is supportive and given the multifaceted nature of their needs is provided most efficiently through multidisciplinary clinics that have shown to prolong survival and improve quality of life. The authors ...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2003
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.0730423100