Cockayne syndrome group B cellular and biochemical functions.
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.
Licht
Cecilie Löe
CL
Laboratory of DNA Repair, Department of Molecular Biology, University of Aarhus, Aarhus, Denmark.
Stevnsner
Tinna
T
Bohr
Vilhelm A
VA
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
D000075223:Poly-ADP-Ribose Binding Proteins; D004265:DNA Helicases; C078673:ERCC6 protein, human; D045643:DNA Repair Enzymes