DNA Excision Repair Protein ERCC-4

DNA Excision Repair Protein ERCC-4, encoded by the ERCC4 gene, is a DNA repair protein. Nucleotide excision repair (NER) involves incision of a DNA strand on each side of a lesion. The two incisions made during NER are catalyzed by separate DNA endonucleases. A tight protein complex formed between ERCC4 and ERCC1 acts as a structure-specific endonuclease for the 5-prime incision during repair. Without DNA damage, this complex moves freely through the nucleus. Ultraviolet light-induced DNA damage causes a transient immobilization of ERCC4/ERCC1 due to engagement of the complex in a single repair event after assembly of individual nucleotide excision repair factors at sites of DNA damage in a distributive fashion. Causative mutations in the ERCC4 gene and strongly reduced levels of encoded protein can be identified in patients with xeroderma pigmentosum type F. (From OMIM and NCI) (NCI Thesaurus)

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