Article
Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis.
Nature communications - 11 Sept 2018
Wang Haibo, Guo Wenting, Mitra Joy, Hegde Pavana M, Vandoorne Tijs, Eckelmann Bradley J, Mitra Sankar, Tomkinson Alan E, Van Den Bosch Ludo, Hegde Muralidhar L
Abstract excerpt
Genome damage and defective repair are etiologically linked to neurodegeneration. However, the specific mechanisms involved remain enigmatic. Here, we identify defects in DNA nick ligation and oxidative damage repair in a subset of amyotrophic lateral sclerosis (ALS) patients. These defects are caused by mutations in the RNA/DNA-binding protein FUS. In healthy neurons, FUS protects the genome by facilitating...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
