Restoration of G1 chemo/radioresistance and double-strand-break repair proficiency by wild-type but not endonuclease-deficient Artemis

Nucleic Acids Res. 2011 Aug;39(15):6500-10. doi: 10.1093/nar/gkr257. Epub 2011 Apr 29.

Abstract

Deficiency in Artemis is associated with lack of V(D)J recombination, sensitivity to radiation and radiomimetic drugs, and failure to repair a subset of DNA double-strand breaks (DSBs). Artemis harbors an endonuclease activity that trims both 5'- and 3'-ends of DSBs. To examine whether endonucleolytic trimming of terminally blocked DSBs by Artemis is a biologically relevant function, Artemis-deficient fibroblasts were stably complemented with either wild-type Artemis or an endonuclease-deficient D165N mutant. Wild-type Artemis completely restored resistance to γ-rays, bleomycin and neocarzinostatin, and also restored DSB-repair proficiency in G0/G1 phase as measured by pulsed-field gel electrophoresis and repair focus resolution. In contrast, cells expressing the D165N mutant, even at very high levels, remained as chemo/radiosensitive and repair deficient as the parental cells, as evidenced by persistent γ-H2AX, 53BP1 and Mre11 foci that slowly increased in size and ultimately became juxtaposed with promyelocytic leukemia protein nuclear bodies. In normal fibroblasts, overexpression of wild-type Artemis increased radioresistance, while D165N overexpression conferred partial repair deficiency following high-dose radiation. Restoration of chemo/radioresistance by wild-type, but not D165N Artemis suggests that the lack of endonucleolytic trimming of DNA ends is the principal cause of sensitivity to double-strand cleaving agents in Artemis-deficient cells.

Publication types

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

MeSH terms

  • Bleomycin / toxicity
  • Cell Survival / drug effects
  • Cell Survival / radiation effects
  • DNA Breaks, Double-Stranded*
  • DNA Repair*
  • DNA-Binding Proteins
  • Endodeoxyribonucleases / deficiency
  • Endodeoxyribonucleases / genetics*
  • Endodeoxyribonucleases / metabolism
  • Endonucleases
  • G1 Phase
  • Humans
  • Mutation
  • Nuclear Proteins / analysis
  • Nuclear Proteins / deficiency
  • Nuclear Proteins / genetics*
  • Nuclear Proteins / metabolism
  • Promyelocytic Leukemia Protein
  • Radiation Tolerance*
  • Transcription Factors / analysis
  • Tumor Suppressor Proteins / analysis
  • Zinostatin / toxicity

Substances

  • DNA-Binding Proteins
  • Nuclear Proteins
  • Promyelocytic Leukemia Protein
  • Transcription Factors
  • Tumor Suppressor Proteins
  • Bleomycin
  • PML protein, human
  • Zinostatin
  • DCLRE1C protein, human
  • Endodeoxyribonucleases
  • Endonucleases