Article
The DDB1-CUL4ADDB2 ubiquitin ligase is deficient in xeroderma pigmentosum group E and targets histone H2A at UV-damaged DNA sites.
Proceedings of the National Academy of Sciences of the United States of America - 21 Feb 2006
Kapetanaki Maria G, Guerrero-Santoro Jennifer, Bisi Dawn C, Hsieh Ching L, Rapić-Otrin Vesna, Levine Arthur S
Abstract excerpt
Xeroderma pigmentosum (XP) is a heritable human disorder characterized by defects in nucleotide excision repair (NER) and the development of skin cancer. Cells from XP group E (XP-E) patients have a defect in the UV-damaged DNA-binding protein complex (UV-DDB), involved in the damage recognition step of NER. UV-DDB comprises two subunits, products of the DDB1 and DDB2 genes, respectively. Mutations in the DDB2...
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