The role of Cockayne Syndrome group B (CSB) protein in base excision repair and aging.
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.
Danish Centre for Molecular Gerontology, Department of Molecular Biology, University of Aarhus, C.F. Møllers Allé, Aarhus C, Denmark. tvs@mb.au.dk
Tinna
T
Stevnsner
Meltem
M
Muftuoglu
Maria Diget
MD
Aamann
Vilhelm A
VA
Bohr
D000375:Aging / Q000235:genetics*; D019588:Aging, Premature; D002843:Chromatin / Q000737:chemistry; D003057:Cockayne Syndrome / Q000235:genetics; D004249:DNA Damage; D004265:DNA Helicases / Q000235:genetics / Q000502:physiology*; D004260:DNA Repair; D045643:DNA Repair Enzymes / Q000235:genetics / Q000502:physiology*; D006801:Humans; D000075223:Poly-ADP-Ribose Binding Proteins
D002843:Chromatin; D000075223:Poly-ADP-Ribose Binding Proteins; D004265:DNA Helicases; C078673:ERCC6 protein, human; D045643:DNA Repair Enzymes