کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3056398 1186565 2009 7 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease
موضوعات مرتبط
علوم زیستی و بیوفناوری علم عصب شناسی عصب شناسی
پیش نمایش صفحه اول مقاله
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease
چکیده انگلیسی

In neurons, cytoplasmic dynein functions as a molecular motor responsible for retrograde axonal transport. An impairment of axonal transport is thought to play a key role in the pathogenesis of neurodegenerative diseases such as amyotrophic lateral sclerosis, the most frequent motor neuron disease in the elderly. In this regard, previous studies described two heterozygous mouse strains bearing missense point mutations in the dynein heavy chain 1 gene that were reported to display late-onset progressive motor neuron degeneration. Here we show, however, that one of these mutant strains, the so-called Cra mice does not suffer from motor neuron loss, even in aged animals. Consistently, we did not observe electrophysiological or biochemical signs of muscle denervation, indicative of motor neuron disease. The «hindlimb clasping» phenotype of Cra mice could rather be due to the prominent degeneration of sensory neurons associated with a loss of muscle spindles. Altogether, these findings show that dynein heavy chain mutation triggers sensory neuropathy rather than motor neuron disease.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Experimental Neurology - Volume 215, Issue 1, January 2009, Pages 146–152
نویسندگان
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