ERCC2 ATP-dependent 5'-3' DNA helicase, component of the general transcription and DNA repair factor IIH (TFIIH) core complex, which is involved in general and transcription-coupled nucleotide excision repair (NER) of damaged DNA and, when complexed to CAK, in RNA transcription by RNA polymerase II. In NER, TFIIH acts by opening DNA around the lesion to allow the excision of the damaged oligonucleotide and its replacement by a new DNA fragment. The ATP-dependent helicase activity of XPD/ERCC2 is required for DNA opening. In transcription, TFIIH has an essential role in transcription initiation. When the pre-initiation complex (PIC) has been established, TFIIH is required for promoter opening and promoter escape. Phosphorylation of the C-terminal tail (CTD) of the largest subunit of RNA polymerase II by the kinase module CAK controls the initiation of transcription. XPD/ERCC2 acts by forming a bridge between CAK and the core-TFIIH complex. Involved in the regulation of vitamin-D receptor activity. As part of the mitotic spindle-associated MMXD complex it plays a role in chromosome segregation. Might 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. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Helicase
Chromosomal Location of Human Ortholog: 19q13.32
Cellular Component:  CAK-ERCC2 complex; cytoplasm; cytosol; MMXD complex; nucleoplasm; nucleus; spindle; transcription factor TFIID complex; transcription factor TFIIH core complex; transcription factor TFIIH holo complex
Molecular Function:  4 iron, 4 sulfur cluster binding; 5'-3' DNA helicase activity; ATP binding; DNA-dependent ATPase activity; metal ion binding; protein binding; protein binding, bridging; protein C-terminus binding; protein N-terminus binding
Biological Process:  7-methylguanosine mRNA capping; aging; apoptotic process; bone mineralization; cell proliferation; central nervous system myelin formation; chromosome segregation; embryonic cleavage; embryonic organ development; erythrocyte maturation; extracellular matrix organization; global genome nucleotide-excision repair; hair cell differentiation; hair follicle maturation; hematopoietic stem cell differentiation; in utero embryonic development; multicellular organism growth; 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 DNA binding; positive regulation of transcription by RNA polymerase II; positive regulation of transcription, DNA-templated; post-embryonic development; protein phosphorylation; regulation of mitotic cell cycle phase transition; response to hypoxia; response to oxidative stress; spinal cord development; termination of RNA polymerase I transcription; transcription by RNA polymerase II; transcription elongation from RNA polymerase I promoter; transcription elongation 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; viral process
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; General transcription and DNA repair factor IIH helicase subunit XPD; 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, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene