The role of CSA and CSB protein in the oxidative stress response

Mech Ageing Dev. 2013 May-Jun;134(5-6):261-9. doi: 10.1016/j.mad.2013.03.006. Epub 2013 Apr 3.


Cockayne syndrome (CS) is a rare hereditary disorder in which infants suffer severe developmental and neurological alterations and early death. Two genes encoding RNA polymerase II cofactors, CSA and CSB, are mutated in this syndrome. CSA and CSB proteins are known to be involved in the transcription-coupled DNA repair pathway but the sensitivity of mutant cells to a number of physical/chemical agents besides UV radiation, such as ionizing radiation, hydrogen peroxide and bioenergetic inhibitors indicate that these proteins play a pivotal role in additional pathways. In this review we will discuss the evidence that implicate CS proteins in the control of oxidative stress response with special emphasis on recent findings that show an altered redox balance and dysfunctional mitochondria in cells derived from patients. Working models of how these new functions might be key to developmental and neurological disease in CS will be discussed.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Cockayne Syndrome / genetics
  • Cockayne Syndrome / metabolism*
  • Cockayne Syndrome / pathology
  • DNA Helicases / genetics
  • DNA Helicases / metabolism*
  • DNA Repair Enzymes / genetics
  • DNA Repair Enzymes / metabolism*
  • DNA Repair*
  • Humans
  • Mitochondria / genetics
  • Mitochondria / metabolism
  • Oxidative Stress*
  • Poly-ADP-Ribose Binding Proteins
  • RNA Polymerase II / genetics
  • RNA Polymerase II / metabolism*
  • Radiation, Ionizing
  • Transcription Factors / genetics
  • Transcription Factors / metabolism*
  • Ultraviolet Rays / adverse effects


  • ERCC8 protein, human
  • Poly-ADP-Ribose Binding Proteins
  • Transcription Factors
  • RNA Polymerase II
  • DNA Helicases
  • ERCC6 protein, human
  • DNA Repair Enzymes