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GeneQ17917471· pop 5· linked from 69 articles

Also known as ARMD5, CKN2, COFS, COFS1, CSB, RAD26, UVSS1, POF11

DNA excision repair protein ERCC-6 (also CS-B protein) is a protein that in humans is encoded by the ERCC6 gene. The ERCC6 gene is located on the long arm of chromosome 10 at position 11.23.

Gene data

ERCC6
Name
ERCC excision repair 6, chromatin remodeling factor
Type
protein-coding
Aliases
ARMD5, CKN2, COFS, COFS1, CSB, CSB-PGBD3, POF11, RAD26, UVSS1

This gene encodes a DNA-binding protein that is important in transcription-coupled excision repair. The encoded protein has ATP-stimulated ATPase activity, interacts with several transcription and excision repair proteins, and may promote complex formation at DNA repair sites. Mutations in this gene are associated with Cockayne syndrome type B and cerebrooculofacioskeletal syndrome 1. Alternative splicing occurs between a splice site from exon 5 of this gene to the 3' splice site upstream of the open reading frame (ORF) of the adjacent gene, piggyback-derived-3 (GeneID:267004), which activates the alternative polyadenylation site downstream of the piggyback-derived-3 ORF. The resulting transcripts encode a fusion protein that shares sequence with the product of each individual gene. [provided by RefSeq, Mar 2016].

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Wikidata facts

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ERCC6 protein.png
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HomoloGene ID
133552
Sources (3)

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~8 min read

Article

10 sections
Contents
  • Function
  • Structure and mechanism
  • Interactions
  • Neurogenesis and neural differentiation
  • Cockayne syndrome
  • DNA repair
  • Implications in cancer
  • References
  • Further reading
  • External links

DNA excision repair protein ERCC-6 (also CS-B protein) is a protein that in humans is encoded by the ERCC6 gene. The ERCC6 gene is located on the long arm of chromosome 10 at position 11.23.

Having 1 or more copies of a mutated ERCC6 causes Cockayne syndrome, type II.

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