Neuronal Intermediate Filaments and Als: a New Look at an Old Question.
From: Department of Laboratory Medicine and Pathobiology, Centre for Research in Neurodegenerative Diseases, University of Toronto, Tanz Neuroscience Building, 6, Queen’s Park Crescent West, Toronto, ON, Canada M5S 3H2.
Biochimica et biophysica acta
- Publish Date:
- ISSN: 0006-3002
- Volume: 1762
- Issue: 11-12
- Pages: 1001-12
- Medium: Print
- Language: English
- Citation (JAMA): Xiao Shangxi, McLean Jesse, Robertson Janice, et al. Neuronal Intermediate Filaments and Als: a New Look at an Old Question.. Biochim. Biophys. Acta ;1762:1001-12
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.
Mesh Headings (Keywords): Amyotrophic Lateral Sclerosis, Animals, Humans, Inclusion Bodies, Intermediate Filament Proteins, Intermediate Filaments, Membrane Glycoproteins, Mice, Mice, Transgenic, Motor Neuron Disease, Motor Neurons, Nerve Tissue Proteins, Neurofilament Proteins, Phosphorylation
Check for Full Text / PubMed Unique Identifier (PMID): 17045786
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