PMID:15132161

Citation	Beaulieu, JM, Nguyen, MD and Julien, JP (1999) Late onset of motor neurons in mice overexpressing wild-type peripherin. J. Cell Biol. 147:531-44
Abstract	Peripherin, a type III intermediate filament (IF) protein, upregulated by injury and inflammatory cytokines, is a component of IF inclusion bodies associated with degenerating motor neurons in sporadic amyotrophic lateral sclerosis (ALS). We report here that sustained overexpression of wild-type peripherin in mice provokes massive and selective degeneration of motor axons during aging. Remarkably, the onset of peripherin-mediated disease was precipitated by a deficiency of neurofilament light (NF-L) protein, a phenomenon associated with sporadic ALS. In NF-L null mice, the overexpression of peripherin led to early- onset formation of IF inclusions and to the selective death of spinal motor neurons at 6 mo of age. We also report the formation of similar peripherin inclusions in presymptomatic transgenic mice expressing a mutant form of superoxide dismutase linked to ALS. Taken together, these results suggest that IF inclusions containing peripherin may play a contributory role in motor neuron disease.
Links	PubMed PMC2151189
Keywords	Aging/genetics; Aging/metabolism; Aging/pathology; Animals; Cell Death/genetics; Intermediate Filament Proteins/genetics; Intermediate Filament Proteins/physiology; Membrane Glycoproteins/genetics; Membrane Glycoproteins/physiology; Mice; Mice, Knockout; Mice, Transgenic; Motor Neurons/pathology; Motor Neurons/physiology; Nerve Degeneration/genetics; Nerve Degeneration/pathology; Nerve Tissue Proteins/genetics; Nerve Tissue Proteins/physiology; Up-Regulation
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