Ontology highlight
ABSTRACT:
SUBMITTER: Elden AC
PROVIDER: S-EPMC2965417 | biostudies-literature | 2010 Aug
REPOSITORIES: biostudies-literature
Nature 20100801 7310
The causes of amyotrophic lateral sclerosis (ALS), a devastating human neurodegenerative disease, are poorly understood, although the protein TDP-43 has been suggested to have a critical role in disease pathogenesis. Here we show that ataxin 2 (ATXN2), a polyglutamine (polyQ) protein mutated in spinocerebellar ataxia type 2, is a potent modifier of TDP-43 toxicity in animal and cellular models. ATXN2 and TDP-43 associate in a complex that depends on RNA. In spinal cord neurons of ALS patients, A ...[more]