title: Cockayne syndrome group B cellular and biochemical functions.
abstract: The devastating genetic disorder Cockayne syndrome (CS) arises from mutations in the CSA and CSB genes. CS is characterized by progressive multisystem degeneration and is classified as a segmental premature-aging syndrome. The CS complementation group B (CSB) protein is at the interface of transcription and DNA repair and is involved in transcription-coupled and global genome-DNA repair, as well as in general transcription. Recent structure-function studies indicate a process-dependent variation in the molecular mechanism employed by CSB and provide a starting ground for a description of the mechanisms and their interplay.
authors.affiliation: Laboratory of DNA Repair, Department of Molecular Biology, University of Aarhus, Aarhus, Denmark.
authors.forename: Cecilie Löe
authors.initials: CL
authors.lastname: Licht
authors.forename: Tinna
authors.initials: T
authors.lastname: Stevnsner
authors.forename: Vilhelm A
authors.initials: VA
authors.lastname: Bohr
mesh_terms: D002874:Chromosome Mapping; D003057:Cockayne Syndrome / Q000235:genetics* / Q000503:physiopathology; D004265:DNA Helicases / Q000235:genetics* / Q000502:physiology; D004260:DNA Repair / Q000235:genetics* / Q000502:physiology; D045643:DNA Repair Enzymes; D006801:Humans; D008957:Models, Genetic; D000075223:Poly-ADP-Ribose Binding Proteins; D014158:Transcription, Genetic / Q000235:genetics* / Q000502:physiology
chemical_list: D000075223:Poly-ADP-Ribose Binding Proteins; D004265:DNA Helicases; C078673:ERCC6 protein, human; D045643:DNA Repair Enzymes