Mech Ageing Dev. 2008 Jul-Aug;129(7-8):441-8. Epub 2008 Apr 30.

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

Stevnsner T, Muftuoglu M, Aamann MD, Bohr VA.

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Danish Centre for Molecular Gerontology, Department of Molecular Biology, University of Aarhus, C.F. Møllers Allé, Aarhus C, Denmark. tvs@mb.au.dk

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.

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