Taxonomic distribution of proteins containing XPGI domains

The tree below includes only several representative species and genera. The complete taxonomic breakdown of all proteins containing XPGI domains can be accessed here. Click the counts or percentage values to display the corresponding proteins.

Defective transcription-coupled repair of oxidative base damage in Cockayne syndrome patients from XP group G.

Cooper PK, Nouspikel T, Clarkson SG, Leadon SA
Science. 1997; 275: 990-3

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

Xeroderma pigmentosum and nucleotide excision repair of DNA.

Tanaka K, Wood RD
Trends Biochem Sci. 1994; 19: 83-6

Nucleotide excision repair is a versatile strategy for removing DNA damage from the genome. Tremendous progress in understanding this process has been made in the last few years, and the field continues to develop rapidly. Exciting connections have emerged between nucleotide excision repair, transcription, and DNA replication, but many mysteries remain concerning the biochemical details of the mechanism, the connection with several human inherited syndromes, and the role of DNA repair in preventing cancer.