Department of Radiation Oncology, UT Southwestern Medical Center, Dallas, TX75390, USA.
Department of Environmental Health, Harvard T.H. Chan School of Public Health, Boston, MA02115, USA.
Nucleic Acids Res. 2023 Jul 21;51(13):6770-6783. doi: 10.1093/nar/gkad505.
Ataxia-telangiectasia mutated (ATM) drives the DNA damage response via modulation of multiple signal transduction and DNA repair pathways. Previously, ATM activity was implicated in promoting the non-homologous end joining (NHEJ) pathway to repair a subset of DNA double-stranded breaks (DSBs), but how ATM performs this function is still unclear. In this study, we identified that ATM phosphorylates the DNA-dependent protein kinase catalytic subunit (DNA-PKcs), a core NHEJ factor, at its extreme C-terminus at threonine 4102 (T4102) in response to DSBs. Ablating phosphorylation at T4102 attenuates DNA-PKcs kinase activity and this destabilizes the interaction between DNA-PKcs and the Ku-DNA complex, resulting in decreased assembly and stabilization of the NHEJ machinery at DSBs. Phosphorylation at T4102 promotes NHEJ, radioresistance, and increases genomic stability following DSB induction. Collectively, these findings establish a key role for ATM in NHEJ-dependent repair of DSBs through positive regulation of DNA-PKcs.
共济失调毛细血管扩张突变基因(ATM)通过调节多种信号转导和 DNA 修复途径来驱动 DNA 损伤反应。先前,ATM 活性被认为可促进非同源末端连接(NHEJ)途径修复部分 DNA 双链断裂(DSB),但 ATM 如何执行此功能尚不清楚。在这项研究中,我们发现 ATM 在 DNA 依赖性蛋白激酶催化亚基(DNA-PKcs)的极端 C 末端丝氨酸 4102(T4102)处发生磷酸化,以响应 DSB。在 T4102 处磷酸化的缺失会减弱 DNA-PKcs 的激酶活性,从而破坏 DNA-PKcs 与 Ku-DNA 复合物之间的相互作用,导致 NHEJ 机制在 DSB 处的组装和稳定减少。T4102 的磷酸化促进了 NHEJ、放射抗性,并在 DSB 诱导后增加了基因组稳定性。总的来说,这些发现确立了 ATM 通过正向调节 DNA-PKcs 在依赖 NHEJ 的 DSB 修复中的关键作用。