Defective Transcription-Coupled Repair of Oxidative Base Damage in Cockayne Syndrome Patients from XP Group G

作者: Priscilla K. Cooper , Thierry Nouspikel , Stuart G. Clarkson , Steven A. Leadon

DOI: 10.1126/SCIENCE.275.5302.990

关键词:

摘要: In normal human cells, damage due to ultraviolet light is preferentially removed from active genes by nucleotide excision repair (NER) in a transcription-coupled (TCR) process that requires the gene products defective Cockayne syndrome (CS). Oxidative damage, including thymine glycols, shown be TCR cells individuals and xeroderma pigmentosum (XP)-A, XP-F, XP-G patients who have NER defects but not severe CS. Thus, of oxidative an XPG function distinct its endonuclease activity. These results raise possibility contributes developmental associated with

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