کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4323065 1291750 2006 21 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی علوم اعصاب سلولی و مولکولی
پیش نمایش صفحه اول مقاله
ALS: A Disease of Motor Neurons and Their Nonneuronal Neighbors
چکیده انگلیسی

Amyotrophic lateral sclerosis is a late-onset progressive neurodegenerative disease affecting motor neurons. The etiology of most ALS cases remains unknown, but 2% of instances are due to mutations in Cu/Zn superoxide dismutase (SOD1). Since sporadic and familial ALS affects the same neurons with similar pathology, it is hoped that therapies effective in mutant SOD1 models will translate to sporadic ALS. Mutant SOD1 induces non-cell-autonomous motor neuron killing by an unknown gain of toxicity. Selective vulnerability of motor neurons likely arises from a combination of several mechanisms, including protein misfolding, mitochondrial dysfunction, oxidative damage, defective axonal transport, excitotoxicity, insufficient growth factor signaling, and inflammation. Damage within motor neurons is enhanced by damage incurred by nonneuronal neighboring cells, via an inflammatory response that accelerates disease progression. These findings validate therapeutic approaches aimed at nonneuronal cells.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: - Volume 52, Issue 1, 5 October 2006, Pages 39–59
نویسندگان
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