The cockayne syndrome group B gene product is involved in cellular repair of 8-hydroxyadenine in DNA.
作者： Jingsheng Tuo , Pawel Jaruga , Henry Rodriguez , Miral Dizdaroglu , Vilhelm A. Bohr
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摘要: Cockayne syndrome (CS) is a human disease characterized by sensitivity to sunlight, severe neurological abnormalities, and accelerated aging. CS has two complementation groups, CS-A CS-B. The CSB gene encodes the protein with 1493 amino acids. We previously reported that involved in cellular repair of 8-hydroxyguanine, an abundant lesion oxidatively damaged DNA putative helicase motif V/VI may play role this process. present study investigated 8-hydroxyadenine (8-OH-Ade), another DNA. Extracts CS-B-null cells mutant site-directed mutation VI domain incised vitro less efficiently than wild type cells. Furthermore, accumulated more their genomic after exposure γ-radiation at doses 2 or 5 Gy. These results suggest contributes 8-OH-Ade required for activity.
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