Ontology highlight
ABSTRACT:
SUBMITTER: Chow HM
PROVIDER: S-EPMC6400560 | biostudies-literature | 2019 Mar
REPOSITORIES: biostudies-literature
The Journal of cell biology 20190114 3
Ataxia-telangiectasia (A-T) is an autosomal recessive disease caused by mutation of the <i>ATM</i> gene and is characterized by loss of cerebellar Purkinje cells, neurons with high physiological activity and dynamic ATP demands. Here, we show that depletion of ATP generates reactive oxygen species that activate ATM. We find that when ATM is activated by oxidative stress, but not by DNA damage, ATM phosphorylates NRF1. This leads to NRF1 dimerization, nuclear translocation, and the up-regulation ...[more]