Sense and sensibility: ATM oxygen stress signaling manages brain cell energetics

Abstract

The ataxia-telangiectasia mutated (ATM) gene regulates DNA damage repair, oxidative stress, and mitochondrial processes. In this issue, Chow et al. (2019. J. Cell Biol. https://doi.org/10.1083/jcb.201806197) connects ATM's oxidative stress response functions to the sensing of metabolic ATP energetics distinctively important in high energy-demanding Purkinje brain cells, which could explain the most distinct A-T patient feature, cerebellar ataxia.