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- W2082055400 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." @default.
- W2082055400 created "2016-06-24" @default.
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- W2082055400 date "1999-11-01" @default.
- W2082055400 modified "2023-09-25" @default.
- W2082055400 title "Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type Peripherin" @default.
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- W2082055400 doi "https://doi.org/10.1083/jcb.147.3.531" @default.
- W2082055400 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2151189" @default.
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