ATR Protects the Genome against R Loops through a MUS81-Triggered Feedback Loop

Mol Cell. 2020 Feb 6;77(3):514-527.e4. doi: 10.1016/j.molcel.2019.10.010. Epub 2019 Nov 7.

Abstract

R loops arising during transcription induce genomic instability, but how cells respond to the R loop-associated genomic stress is still poorly understood. Here, we show that cells harboring high levels of R loops rely on the ATR kinase for survival. In response to aberrant R loop accumulation, the ataxia telangiectasia and Rad3-related (ATR)-Chk1 pathway is activated by R loop-induced reversed replication forks. In contrast to the activation of ATR by replication inhibitors, R loop-induced ATR activation requires the MUS81 endonuclease. ATR protects the genome from R loops by suppressing transcription-replication collisions, promoting replication fork recovery, and enforcing a G2/M cell-cycle arrest. Furthermore, ATR prevents excessive cleavage of reversed forks by MUS81, revealing a MUS81-triggered and ATR-mediated feedback loop that fine-tunes MUS81 activity at replication forks. These results suggest that ATR is a key sensor and suppressor of R loop-induced genomic instability, uncovering a signaling circuitry that safeguards the genome against R loops.

Keywords: ATR; Chk1; Fork reversal; Genomic Instability; MUS81; R loop; checkpoint.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Ataxia Telangiectasia Mutated Proteins / metabolism*
  • Ataxia Telangiectasia Mutated Proteins / physiology
  • Cell Cycle Proteins / metabolism
  • Checkpoint Kinase 1 / genetics
  • DNA Damage
  • DNA Repair
  • DNA Replication / genetics
  • DNA Replication / physiology
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism*
  • Endonucleases / genetics
  • Endonucleases / metabolism*
  • Genomic Instability / physiology
  • HeLa Cells
  • Humans
  • Phosphorylation
  • Protein Kinases / metabolism
  • R-Loop Structures / genetics*
  • Signal Transduction

Substances

  • Cell Cycle Proteins
  • DNA-Binding Proteins
  • Protein Kinases
  • ATR protein, human
  • Ataxia Telangiectasia Mutated Proteins
  • Checkpoint Kinase 1
  • Endonucleases
  • MUS81 protein, human