The neurodegeneration found in human ataxia-telangiectasia (A-T) is caused by mutations in the ATM (A-T mutated) gene. Li et al. have identified the polycomb group protein with histone methyltransferase activity called EZH2 as a target of the ATM kinase. The study shows that ATM deficiency increases EZH2 stability, thus increasing methylated histone marks. This results in epigenetic changes in transcription that compromise the health and survival of CNS neurons.
- Jiali Li
- Ronald P Hart
- Karl Herrup