Ontology highlight
ABSTRACT:
SUBMITTER: Beaulieu JM
PROVIDER: S-EPMC2151189 | biostudies-literature | 1999 Nov
REPOSITORIES: biostudies-literature
Beaulieu J M JM Nguyen M D MD Julien J P JP
The Journal of cell biology 19991101 3
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 (N ...[more]