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lüll XPG: its products and biological roles Scharer ODAdv Exp Med Biol 2008[]; 637 (ä): 83-92Xeroderma pigmetosum patients of the complementation group G are rare. One group of XP-G patients displays a rather mild and typical XP phenotype. Mutations in these patients interfere with the function of XPG in the nucleotide excision repair, where it has a structural role in the assembly of the preincision complex and a catalytic role in making the incision 3' to the damaged site in DNA. Another set of XP-G patient is much more severely affected, displaying combined symptoms of xeroderma pigmentosum and Cockayne syndrome, referred to as XP/CS complex. Although the molecular basis leading to the XP/CS complex has not yet been fully established, current evidence suggests that these patients suffer from a mild defect in transcription in addition to a repair defect. Here, the history of how the XPG gene was discovered, the biochemical properties of the XPG protein and the molecular defects found in XP-G patients and mouse models are reviewed.|Animals[MESH]|Cockayne Syndrome/genetics[MESH]|DNA Repair[MESH]|DNA-Binding Proteins/*physiology[MESH]|Disease Models, Animal[MESH]|Endonucleases/*physiology[MESH]|Humans[MESH]|Nuclear Proteins/*physiology[MESH]|Transcription Factors/*physiology[MESH]|Xeroderma Pigmentosum/genetics[MESH] |