Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
1905885 | Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | 2006 | 12 Pages |
Abstract
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.
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Authors
Shangxi Xiao, Jesse McLean, Janice Robertson,