ERCC6 Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the at sites of RNA polymerase II-blocking lesions. Belongs to the SNF2/RAD54 helicase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: DNA repair, damage; EC 3.6.1.-; EC 3.6.4.-; Helicase; Transcription regulation
Chromosomal Location of Human Ortholog: 10q11.23
Cellular Component:  nucleolus; nucleoplasm; nucleus; transcription elongation factor complex
Molecular Function:  ATP binding; chromatin binding; DNA binding; DNA-dependent ATPase activity; protein binding; protein C-terminus binding; protein N-terminus binding; protein tyrosine kinase activator activity; protein-containing complex binding; sequence-specific DNA binding
Biological Process:  activation of JNKK activity; activation of JUN kinase activity; base-excision repair; DNA duplex unwinding; intrinsic apoptotic signaling pathway in response to DNA damage; multicellular organism growth; photoreceptor cell maintenance; positive regulation of defense response to virus by host; positive regulation of DNA repair; positive regulation of DNA-templated transcription, elongation; positive regulation of gene expression; positive regulation of gene expression, epigenetic; positive regulation of peptidyl-serine phosphorylation of STAT protein; positive regulation of protein tyrosine kinase activity; pyrimidine dimer repair; regulation of DNA-templated transcription, elongation; response to gamma radiation; response to oxidative stress; response to superoxide; response to toxic substance; response to UV; response to UV-B; response to X-ray; transcription by RNA polymerase II; transcription elongation from RNA polymerase I promoter; transcription-coupled nucleotide-excision repair
Disease: Cerebrooculofacioskeletal Syndrome 1; Cockayne Syndrome B; De Sanctis-cacchione Syndrome; Lung Cancer; Macular Degeneration, Age-related, 5; Premature Ovarian Failure 11; Uv-sensitive Syndrome 1
Reference #:  Q03468 (UniProtKB)
Alt. Names/Synonyms: ARMD5; ATP-dependent helicase ERCC6; Chimeric CSB-PGBD3 protein; Chimeric ERCC6-PGBD3 protein; CKN2; Cockayne syndrome B protein; Cockayne syndrome group B protein; Cockayne syndrome protein CSB; COFS; COFS1; CSB; CSB-PGBD3; DNA excision repair protein ERCC-6; ERCC excision repair 6, chromatin remodeling factor; ERCC6; ERCC6-PGBD3 fusion protein; excision repair cross-complementation group 6; excision repair cross-complementing rodent repair deficiency, complementation group 6; POF11; RAD26; Rad26 homolog; UVSS1
Gene Symbols: ERCC6
Molecular weight: 168,416 Da
Basal Isoelectric point: 8.28  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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