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Details on Person UniProt:P19447 ERCC3

Class:Id	ReferenceGeneProduct:67440
_chainChangeLog	chain:1-782 added on Sat February 7 2015
_displayName	UniProt:P19447 ERCC3
_timestamp	2025-02-21 19:37:35
chain	chain:1-782
checksum	F5F4D3A89A7DF826
comment	FUNCTION ATP-dependent 3'-5' DNA helicase/translocase (PubMed:17466626, PubMed:27193682, PubMed:33902107, PubMed:8465201, PubMed:8663148). Binds dsDNA rather than ssDNA, unzipping it in a translocase rather than classical helicase activity (PubMed:27193682, PubMed:33902107). Component of the general transcription and DNA repair factor IIH (TFIIH) core complex (PubMed:10024882, PubMed:17466626, PubMed:8157004, PubMed:8465201). When complexed to CDK-activating kinase (CAK), involved in RNA transcription by RNA polymerase II. The ATPase activity of XPB/ERCC3, but not its helicase activity, is required for DNA opening; it may wrap around the damaged DNA wedging it open, causing localized melting that allows XPD/ERCC2 helicase to anchor (PubMed:10024882, PubMed:17466626). In transcription, TFIIH has an essential role in transcription initiation (PubMed:30894545, PubMed:8157004). When the pre-initiation complex (PIC) has been established, TFIIH is required for promoter opening and promoter escape (PubMed:8157004). The ATP-dependent helicase activity of XPB/ERCC3 is required for promoter opening and promoter escape (PubMed:10024882). In transcription pre-initiation complexes induces and propagates a DNA twist to open DNA (PubMed:27193682, PubMed:33902107). Also involved in transcription-coupled nucleotide excision repair (NER) of damaged DNA (PubMed:17466626, PubMed:2111438, PubMed:8157004). 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 structure of the TFIIH transcription complex differs from the NER-TFIIH complex; large movements by XPD/ERCC2 and XPB/ERCC3 are stabilized by XPA (PubMed:31253769, PubMed:33902107). XPA retains XPB/ERCC3 at the 5' end of a DNA bubble (mimicking DNA damage) (PubMed:31253769).CATALYTIC ACTIVITY Couples ATP hydrolysis with the unwinding of duplex DNA by translocating in the 3'-5' direction.CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)ACTIVITY REGULATION Phosphorylation on Ser-751 by CK2 controls the 5'-excision activity of ERCC1-XPF endonuclease; phosphorylated protein inhibits the excision activity and thus NER (PubMed:15549133). ATPase activity is stimulated by TFIIH subunit p52 (GTF2H4) (PubMed:17466626). DNA translocase activity by this subunit in TFIIH is stimulated by XPA, ERCC5/XPG and XFP plus ERCC1; translocase activity is sensitive to triptolide which targets this enzyme (PubMed:31253769).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. 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:8663148, PubMed:9852112). Interacts with PUF60 (PubMed:10882074, PubMed:11239393). Interacts with ATF7IP (PubMed:19106100). Interacts with KAT2A; leading to KAT2A recruitment to promoters and acetylation of histones (PubMed:30894545). 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 Phosphorylation on Ser-751 by CK2 controls the 5'-excision activity of ERCC1-XPF endonuclease; phosphorylated protein inhibits the excision activity and thus NER (PubMed:15549133). Dephosphorylation reactivates the 5'-excision step (PubMed:15549133). Phosphorylation has no effect on transcription or the 3'-5' helicase activity (PubMed:15549133).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.MISCELLANEOUS The TFIIH core complex from patient XP11BE (a patient with XP/CS who has a C-terminal splice-site frameshift from residue 741 in this protein) reconstitutes in vitro transcription about 30% less well than wild-type but does not restore NER in vitro or in vivo (PubMed:8663148). The XP11BE TFIIH complex has all the subunits in the same stoichiometry as wild-type (PubMed:8663148). Purified mutant protein has very weak 3'-5' helicase and ATPase activities (PubMed:8663148). In another paper the mutant protein has wild-type 3'-5' helicase activity (PubMed:15549133). The mutation is recessive to wild-type (PubMed:2167179, PubMed:8663148). XPB/ERCC3 is not phosphorylated in vitro in this mutant, and does not restore transcription in XPB/ERCC3-defective cells (PubMed:15549133).MISCELLANEOUS Conventional DNA helicases unwind DNA by binding to a ssDNA overhang of dsDNA and then translocating on this strand with cycles of ATP binding and hydrolysis to 'unzip' the dsDNA. XPB/ERCC3 is believed to be an unconventional DNA helicase principally because it translocates in a 3'-5' direction along dsDNA instead of ssDNA, thus it is referred to as a DNA translocase (PubMed:27193682, PubMed:33902107).SIMILARITY Belongs to the helicase family. RAD25/XPB subfamily.
description	recommendedName: fullName evidence="31"General transcription and DNA repair factor IIH helicase/translocase subunit XPB shortName: TFIIH subunit XPB ecNumber evidence="24 37"5.6.2.4 alternativeName: Basic transcription factor 2 89 kDa subunit shortName: BTF2 p89 alternativeName: fullName evidence="33"DNA 3'-5' helicase/translocase XPB alternativeName: DNA excision repair protein ERCC-3 alternativeName: fullName evidence="30"DNA repair protein complementing XP-B cells alternativeName: fullName evidence="32"TFIIH basal transcription factor complex 89 kDa subunit shortName: TFIIH 89 kDa subunit shortName: TFIIH p89 alternativeName: Xeroderma pigmentosum group B-complementing protein
geneName	ERCC3 XPB XPBC
identifier	P19447
isSequenceChanged	FALSE
keyword	3D-structure ATP-binding Cockayne syndrome Deafness Disease variant DNA damage DNA repair DNA-binding Dwarfism Helicase Host-virus interaction Hydrolase Ichthyosis Isomerase Nucleotide-binding Nucleus Phosphoprotein Proteomics identification Reference proteome Transcription Transcription regulation 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
name	ERCC3
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:8996549] ENSEMBL:ENSG00000163161 ERCC3 [Homo sapiens]
secondaryIdentifier	ERCC3_HUMAN Q53QM0
sequenceLength	782
species	[Species:48887] Homo sapiens
(referenceEntity)	[EntityWithAccessionedSequence:67439] ERCC3 [nucleoplasm] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P19447 ERCC3 (67440)