کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3070798 1580742 2007 13 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Differential expression of molecular motors in the motor cortex of sporadic ALS
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی عصب شناسی
پیش نمایش صفحه اول مقاله
Differential expression of molecular motors in the motor cortex of sporadic ALS
چکیده انگلیسی

The molecular mechanisms underlying the selective neurodegeneration of motor neurons in amyotrophic lateral sclerosis (ALS) are inadequately understood. Recent breakthroughs have implicated impaired axonal transport, mediated by molecular motors, as a key element for disease onset and progression. The current work identifies the expression of 15 kinesin-like motors in healthy human motor cortex, including three novel isoforms. Our comprehensive quantitative mRNA analysis in control and sporadic ALS (SALS) motor cortex specimens detects SALS-specific down-regulation of KIF1Bβ and novel KIF3Aβ, two isoforms we show to be enriched in the brain, and also of SOD1, a key enzyme linked to familial ALS. This is accompanied by a marked reduction of KIF3Aβ protein levels. In the motor cortex KIF3Aβ localizes in cholinergic neurons, including upper motor neurons. No mutations causing splicing defects or altering protein-coding sequences were identified in the genes of the three proteins. The present study implicates two motor proteins as possible candidates in SALS pathology.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neurobiology of Disease - Volume 26, Issue 3, June 2007, Pages 577–589
نویسندگان
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