Ontology highlight
ABSTRACT:
SUBMITTER: Yang D
PROVIDER: S-EPMC4575383 | biostudies-literature | 2015 Oct
REPOSITORIES: biostudies-literature
Yang Dejun D Abdallah Abbas A Li Zhaodong Z Lu Yubing Y Almeida Sandra S Gao Fen-Biao FB
Acta neuropathologica 20150602 4
C9ORF72 repeat expansion is the most common genetic mutation in frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Abnormal dipeptide repeat proteins (DPRs) generated from repeat-associated non-AUG (RAN) translation of repeat-containing RNAs are thought to be pathogenic; however, the mechanisms are unknown. Here we report that (GR)80 and (PR)80 are toxic in neuronal and non-neuronal cells in Drosophila. In contrast to reported shorter poly(GR) forms, (GR)80 is mostly localize ...[more]