The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging

Mech Ageing Dev. 2008 Jul-Aug;129(7-8):441-8. doi: 10.1016/j.mad.2008.04.009. Epub 2008 Apr 30.

Abstract

Cockayne Syndrome (CS) is a rare human genetic disorder characterized by progressive multisystem degeneration and segmental premature aging. The CS complementation group B (CSB) protein is engaged in transcription coupled and global nucleotide excision repair, base excision repair and general transcription. However, the precise molecular function of the CSB protein is still unclear. In the current review we discuss the involvement of CSB in some of these processes, with focus on the role of CSB in repair of oxidative damage, as deficiencies in the repair of these lesions may be an important aspect of the premature aging phenotype of CS.

Publication types

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

MeSH terms

  • Aging / genetics*
  • Aging, Premature
  • Chromatin / chemistry
  • Cockayne Syndrome / genetics
  • DNA Damage
  • DNA Helicases / genetics
  • DNA Helicases / physiology*
  • DNA Repair Enzymes / genetics
  • DNA Repair Enzymes / physiology*
  • DNA Repair*
  • Humans
  • Poly-ADP-Ribose Binding Proteins

Substances

  • Chromatin
  • Poly-ADP-Ribose Binding Proteins
  • DNA Helicases
  • ERCC6 protein, human
  • DNA Repair Enzymes