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ERCC2 ATP-dependent 5'-3' DNA helicase, component of the core- TFIIH basal transcription factor. Involved in nucleotide excision repair (NER) of DNA by opening DNA around the damage, and in RNA transcription by RNA polymerase II by anchoring the CDK-activating kinase (CAK) complex, composed of CDK7, cyclin H and MAT1, to the core-TFIIH complex. Involved in the regulation of vitamin-D receptor activity. Might also have a role in aging process and could play a causative role in the generation of skin cancers. Belongs to the helicase family. RAD3/XPD subfamily. One of the six subunits forming the core-TFIIH basal transcription factor which associates with the CAK complex composed of CDK7, CCNH/cyclin H and MNAT1 to form the TFIIH basal transcription factor. The interaction with GTF2H2 results in the stimulation of the 5'-->3' helicase activity. Interacts with Epstein-Barr virus EBNA2. Note: This description may include information from UniProtKB.
Protein type: EC; Helicase
Chromosomal Location of Human Ortholog: 19q13.32
Cellular Component: cyclin-dependent protein kinase activating kinase holoenzyme complex; cytoplasm; cytosol; holo TFIIH complex; nucleoplasm; nucleus; spindle; transcription factor TFIID complex
Molecular Function: 5'-3' DNA helicase activity; DNA-dependent ATPase activity; protein binding; protein C-terminus binding; protein kinase activity; protein N-terminus binding; RNA polymerase subunit kinase activity
Biological Process: apoptosis; chromosome segregation; hair cell differentiation; mRNA capping; nucleotide-excision repair; nucleotide-excision repair, DNA duplex unwinding; nucleotide-excision repair, DNA incision; nucleotide-excision repair, DNA incision, 3'-to lesion; nucleotide-excision repair, DNA incision, 5'-to lesion; nucleotide-excision repair, preincision complex assembly; nucleotide-excision repair, preincision complex stabilization; positive regulation of transcription from RNA polymerase II promoter; positive regulation of transcription, DNA-dependent; response to oxidative stress; RNA elongation from RNA polymerase I promoter; RNA elongation from RNA polymerase II promoter; termination of RNA polymerase I transcription; transcription from RNA polymerase II promoter; transcription initiation from RNA polymerase I promoter; transcription initiation from RNA polymerase II promoter; transcription-coupled nucleotide-excision repair; UV protection
Disease: Cerebrooculofacioskeletal Syndrome 2; Trichothiodystrophy, Photosensitive; Xeroderma Pigmentosum, Complementation Group D
Reference #:  P18074 (UniProtKB)
Alt. Names/Synonyms: Basic transcription factor 2 80 kDa subunit; BTF2 p80; COFS2; CXPD; DNA excision repair protein ERCC-2; DNA repair protein complementing XP-D cells; EM9; ERCC2; excision repair cross-complementing rodent repair deficiency, complementation group 2; MGC102762; MGC126218; MGC126219; TFIIH 80 kDa subunit; TFIIH basal transcription factor complex 80 kDa subunit; TFIIH basal transcription factor complex helicase subunit; TFIIH p80; TTD; xeroderma pigmentosum complementary group D; Xeroderma pigmentosum group D-complementing protein; XPD; XPDC
Gene Symbols: ERCC2
Molecular weight: 86,909 Da
Basal Isoelectric point: 6.72  Predict pI for various phosphorylation states
CST Pathways:  Protein Acetylation
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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