| Literature DB >> 33551183 |
Charlotte M Fare1, James Shorter2.
Abstract
Poly(PR), a toxic dipeptide-repeat protein, translated from the pathogenic G4C2 repeat expansion in C9orf72, contributes to c9 amyotrophic lateral sclerosis/frontotemporal dementia (c9ALS/FTD). However, precisely how poly(PR) elicits neurodegeneration has remained unclear. Maor-Nof et al. now establish that poly(PR) remodels the neuronal epigenome to promote proapoptotic p53 activity involving PUMA, which drives neurodegeneration in several models.Entities:
Keywords: PUMA; TDP-43; c9ALS/FTD; dipeptide-repeat protein; p53; poly(PR)
Year: 2021 PMID： 33551183 DOI： 10.1016/j.tig.2021.01.008
Source DB: PubMed Journal: Trends Genet ISSN： 0168-9525 Impact factor: 11.639