Defective resolution of pH2AX foci and enhanced DNA breakage in ionizing radiation-treated cockayne syndrome B cells

Monica Ropolo, Enrico Cappelli, Mara Foresta, Alessandro Poggi, Luca Proietti-De-Santis, Guido Frosina

Research output: Contribution to journalArticle

Abstract

We have previously shown that DNA repair of oxidized bases (either purines or pyrimidines) is inefficient in cells from patients with Cockayne syndrome (cs), a rare developmental and neurological genetic disorder. Here, we show for the first time that resolution of ionizing radiation (IR)-induced pH2AX foci, an indicator of DNA double-strand breaks, is significantly delayed in IR-treated cells belonging to the B complementation group of cs (csb). Using alkaline single-cell gel electrophoresis, which predominantly detects single-strand breaks, we further demonstrate elevated DNA breakage in csb cells early after irradiation. Both the delayed resolution of pH2AX foci and the early DNA breakage of csb cells were partially complemented by expression of wild-type CSB protein. Hence, the csb mutation impairs resolution of pH2AX foci and causes DNA fragility, broadening the spectrum of lesions whose processing is altered in this syndrome.

Original languageEnglish
Pages (from-to)272-276
Number of pages5
JournalIUBMB Life
Volume63
Issue number4
DOIs
Publication statusPublished - Apr 2011

Keywords

  • Cockayne syndrome
  • comet
  • DNA repair
  • H2AX
  • strand break

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Clinical Biochemistry
  • Molecular Biology
  • Genetics

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