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Title: Ercc6  
Author: World Heritage Encyclopedia
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Subject: P53, Xeroderma pigmentosum, Cockayne syndrome, Nucleotide excision repair, CSB, Chromosome 10 (human), DeSanctis–Cacchione syndrome
Publisher: World Heritage Encyclopedia


Excision repair cross-complementing rodent repair deficiency, complementation group 6
ERCC6 Gene

DNA excision repair protein ERCC-6 is a protein that in humans is encoded by the ERCC6 gene.[1][2][3]

Having two copies of a mutated ERCC6 causes Cockayne syndrome, type II.

The ERCC6 protein is a DNA-binding protein important in transcription-coupled excision repair. The protein has ATP-stimulated ATPase activity; there are contradictory publications reporting presence or absence of helicase activity. The protein appears to interact with several transcription and excision repair proteins, and may promote complex formation at repair sites.[3]


ERCC6 has been shown to interact with P53.[4][5]


Further reading

External links

  • GeneReviews/NCBI/NIH/UW entry on Cockayne syndrome

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