Article
Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predisposition.
Cell - 2 May 1997
van der Horst G T, van Steeg H, Berg R J, van Gool A J, de Wit J, Weeda G, Morreau H, Beems R B, van Kreijl C F, de Gruijl F R, Bootsma D, Hoeijmakers J H
Abstract excerpt
A mouse model for the nucleotide excision repair disorder Cockayne syndrome (CS) was generated by mimicking a truncation in the CSB(ERCC6) gene of a CS-B patient. CSB-deficient mice exhibit all of the CS repair characteristics: ultraviolet (UV) sensitivity, inactivation of transcription-coupled r...
Topics
- Alleles
- Amino Acid Sequence
- Animals
- Cockayne Syndrome
- DNA Helicases
- DNA Repair
- DNA Repair Enzymes
- Disease Models, Animal
- Female
- Humans
- Male
- Mice
- Mice, Inbred C57BL
- Mice, Mutant Strains
- Mutagenesis