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Details on Person UniProt:P18074 ERCC2

Class:Id	ReferenceGeneProduct:67444
_chainChangeLog	chain:1-760 added on Fri February 6 2015
_displayName	UniProt:P18074 ERCC2
_timestamp	2026-02-20 21:29:36
chain	chain:1-760
checksum	746C0888CDF2E331
comment	FUNCTION ATP-dependent 5'-3' DNA helicase (PubMed:31253769, PubMed:8413672, PubMed:9771713). Component of the general transcription and DNA repair factor IIH (TFIIH) core complex, not absolutely essential for minimal transcription in vitro (PubMed:10024882, PubMed:17466626, PubMed:9771713). Required for transcription-coupled nucleotide excision repair (NER) of damaged DNA; recognizes damaged bases (PubMed:17466626, PubMed:23352696, PubMed:9771713). Sequestered in chromatin on UV-damaged DNA (PubMed:23352696). When complexed to CDK-activating kinase (CAK), involved in 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. Involved in DNA lesion verification (PubMed:31253769). 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. The structure of the TFIIH transcription complex differs from the NER-TFIIH complex; large movements by XPD/ERCC2 and XPB/ERCC3 are stabilized by XPA which allow this subunit to contact ssDNA (PubMed:31253769, PubMed:33902107). 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.CATALYTIC ACTIVITY Couples ATP hydrolysis with the unwinding of duplex DNA at the replication fork by translocating in the 5'-3' direction.CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)COFACTOR Binds 1 [4Fe-4S] cluster.ACTIVITY REGULATION Interaction with GTF2H2 (p44) results in stimulation of the 5'-3' helicase activity of this subunit (PubMed:9771713). DNA unwinding by this subunit in TFIIH is stimulated 4-fold by XPA and 20-fold by ERCC5/XPG (PubMed:31253769).BIOPHYSICOCHEMICAL PROPERTIES Optimum pH is 6.5 for both helicase and DNA-stimulated ATPase activity.SUBUNIT Component of the 7-subunit TFIIH core complex composed of XPB/ERCC3, XPD/ERCC2, GTF2H1, GTF2H2, GTF2H3, GTF2H4 and GTF2H5, which is active in NER (PubMed:9771713, PubMed:9852112). The core complex associates with the 3-subunit CDK-activating kinase (CAK) module composed of CCNH/cyclin H, CDK7 and MNAT1 to form the 10-subunit holoenzyme (holo-TFIIH) active in transcription (PubMed:9771713, PubMed:9852112). Interacts with GTF2H2 (p44) which stimulates the 5'-3' helicase activity of this subunit (PubMed:9771713). Component of the MMXD complex, which includes CIAO1, ERCC2, CIAO2B, MMS19 and SLC25A5 (PubMed:20797633). Interacts with CIAO1 and CIAO2B; the interaction WITH CIAO2B is direct (PubMed:23891004). Interacts with ATF7IP (PubMed:19106100). Interacts directly with MMS19 (PubMed:23585563). Part of TBP-based Pol II pre-initiation complex (PIC), in which Pol II core assembles with general transcription factors and other specific initiation factors including GTF2E1, GTF2E2, GTF2F1, GTF2F2, TCEA1, ERCC2, ERCC3, GTF2H2, GTF2H3, GTF2H4, GTF2H5, GTF2A1, GTF2A2, GTF2B and TBP; this large multi-subunit PIC complex mediates DNA unwinding and targets Pol II core to the transcription start site where the first phosphodiester bond forms.SUBUNIT (Microbial infection) Interacts with Epstein-Barr virus EBNA2.INTERACTION Interacts with GTF2H2/p44 via the C-terminus of this protein; mutations in the C-terminal region of XPD do not alter its helicase activity, but prevent its interaction with and helicase stimulation by GTF2H2/p44 (PubMed:9771713).PTM ISGylated.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the helicase family. RAD3/XPD subfamily.
description	recommendedName: General transcription and DNA repair factor IIH helicase subunit XPD shortName: TFIIH subunit XPD ecNumber evidence="22 29 35"5.6.2.3 alternativeName: Basic transcription factor 2 80 kDa subunit shortName: BTF2 p80 alternativeName: CXPD alternativeName: fullName evidence="40"DNA 5'-3' helicase XPD alternativeName: DNA excision repair protein ERCC-2 alternativeName: DNA repair protein complementing XP-D cells alternativeName: TFIIH basal transcription factor complex 80 kDa subunit shortName: TFIIH 80 kDa subunit shortName: TFIIH p80 alternativeName: Xeroderma pigmentosum group D-complementing protein
geneName	ERCC2 XPD XPDC
identifier	P18074
isSequenceChanged	FALSE
keyword	3D-structure 4Fe-4S Alternative splicing ATP-binding Cataract Chromosome partition Cockayne syndrome Cytoplasm Cytoskeleton Deafness Disease variant DNA damage DNA repair DNA-binding Dwarfism Helicase Host-virus interaction Hydrolase Ichthyosis Iron Iron-sulfur Isomerase Magnesium Metal-binding Nucleotide-binding Nucleus Proteomics identification Reference proteome Transcription Transcription regulation Ubl conjugation Xeroderma pigmentosum
modified	[InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9909836] Weiser, Joel, 2024-05-14 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20
name	ERCC2
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:8997148] ENSEMBL:ENSG00000104884 ERCC2 [Homo sapiens]
secondaryIdentifier	ERCC2_HUMAN Q2TB78 Q2YDY2 Q7KZU6 Q8N721
sequenceLength	760
species	[Species:48887] Homo sapiens
(isoformParent)	[ReferenceIsoform:8974265] UniProt:P18074-1 ERCC2 [Homo sapiens] [ReferenceIsoform:8974266] UniProt:P18074-2 ERCC2 [Homo sapiens]
(referenceEntity)	[EntityWithAccessionedSequence:67443] ERCC2 [nucleoplasm] [Homo sapiens] [EntityWithAccessionedSequence:2564770] ERCC2 [cytosol] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P18074 ERCC2 (67444)