A new role for ATM: regulating mitochondrial function and mitophagy.

Journal Article (Journal Article)

The various pathologies in ataxia telangiectasia (A-T) patients including T-cell lymphomagenesis have been attributed to defects in the DNA damage response pathway because ATM, the gene mutated in this disease, is a key mediator of this process. Analysis of Atm-deficient thymocytes in mice reveals that the absence of this gene results in altered mitochondrial homeostasis, a phenomenon that appears to result from abnormal mitophagy engagement. Interestingly, allelic loss of the autophagic gene Becn1 delays tumorigenesis in Atm-null mice presumably by reversing the mitochondrial abnormalities and not by improving the DNA damage response (DDR) pathway. Thus, ATM plays a critical role in modulating mitochondrial homeostasis perhaps by regulating mitophagy.

Full Text

Duke Authors

Cited Authors

  • Valentin-Vega, YA; Kastan, MB

Published Date

  • May 1, 2012

Published In

Volume / Issue

  • 8 / 5

Start / End Page

  • 840 - 841

PubMed ID

  • 22617444

Pubmed Central ID

  • PMC3679088

Electronic International Standard Serial Number (EISSN)

  • 1554-8635

Digital Object Identifier (DOI)

  • 10.4161/auto.19693


  • eng

Conference Location

  • United States