期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
卷 1762, 期 11-12, 页码 1001-1012出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbadis.2006.09.003
关键词
amyotrophic lateral sclerosis; intermediate filament; neurofilament; peripherin
One of the pathological hallmarks of ALS is the presence of axonal spheroids and perikaryal accumulations/aggregations comprised of the neuronal intermediate filament proteins, neurofilaments and peripherin. These abnormalities represent a point of convergence of both familial and sporadic forms of the disease and understanding their formation may reveal shared pathways in what is otherwise considered a highly heterogeneous disorder. Here we provide a review of the basic biology of neurofilaments and peripherin and the evidence linking them with ALS disease pathogenesis. (c) 2006 Elsevier B.V. All rights reserved.
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