UniProt:P28715 ERCC5

chain
  • chain:1-1186
checksum B0A844D617C53F2E
comment
  • FUNCTION Single-stranded structure-specific DNA endonuclease involved in DNA excision repair (PubMed:32522879, PubMed:32821917, PubMed:7651464, PubMed:8078765, PubMed:8090225, PubMed:8206890). Makes the 3'incision in DNA nucleotide excision repair (NER) (PubMed:32522879, PubMed:32821917, PubMed:8078765, PubMed:8090225). Binds and bends DNA repair bubble substrate and breaks base stacking at the single-strand/double-strand DNA junction of the DNA bubble (PubMed:32522879). Plays a role in base excision repair (BER) by promoting the binding of DNA glycosylase NTHL1 to its substrate and increasing NTHL1 catalytic activity that removes oxidized pyrimidines from DNA (PubMed:9927729). Involved in transcription-coupled nucleotide excision repair (TCR) which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes (PubMed:16246722). Functions during the initial step of TCR in cooperation with ERCC6/CSB to recognized stalled RNA polymerase II (PubMed:16246722). Also, stimulates ERCC6/CSB binding to the DNA repair bubble and ERCC6/CSB ATPase activity (PubMed:16246722). Required for DNA replication fork maintenance and preservation of genomic stability (PubMed:26833090, PubMed:32522879). Involved in homologous recombination repair (HRR) induced by DNA replication stress by recruiting RAD51, BRCA2, and PALB2 to the damaged DNA site (PubMed:26833090). In TFIIH stimulates the 5'-3' helicase activity of XPD/ERCC2 and the DNA translocase activity of XPB/ERCC3 (PubMed:31253769). During HRR, binds to the replication fork with high specificity and stabilizes it (PubMed:32522879). Also, acts upstream of HRR, to promote the release of BRCA1 from DNA (PubMed:26833090).COFACTOR Binds 2 magnesium ions per subunit. They probably participate in the reaction catalyzed by the enzyme. May bind an additional third magnesium ion after substrate binding.BIOPHYSICOCHEMICAL PROPERTIES Optimum pH is 6.5-7.SUBUNIT Monomer (PubMed:32522879). Homodimer (PubMed:32522879). Component of the homologous recombination repair (HR) complex composed of ERCC5/XPG, BRCA2, PALB2, DSS1 and RAD51 (PubMed:26833090). Within the complex, interacts with BRCA2 and PALB2 (PubMed:26833090). Interacts with RNA polymerase II (PubMed:16246722). Interacts (via C-terminus) with ERCC6/CSB; the interaction stimulates ERCC6/CSB binding to the DNA repair bubble and ERCC6/CSB ATPase activity (PubMed:16246722). May form a complex composed of RNA polymerase II, ERCC6/CSB and ERCC5/XPG which associates with the DNA repair bubble during transcription-coupled nucleotide excision repair (PubMed:16246722). Interacts with BRCA1; the interaction promotes the release of BRCA1 from DNA (PubMed:26833090). Interacts with PCNA (PubMed:9305916). Interacts with NTHL1; the interaction stimulates NTHL1 activity and NTHL1 binding to its DNA substrate (PubMed:9927729).INTERACTION Colocalizes with RAD51 to nuclear foci in S phase (PubMed:26833090). Localizes to DNA double-strand breaks (DBS) during replication stress (PubMed:26833090). Colocalizes with BRCA2 to nuclear foci following DNA replication stress (PubMed:26833090).ALTERNATIVE PRODUCTS Induced by replication stress caused by DNA double-strand breaks (DBS).DOMAIN Both nuclear localization signals 1 and 2 act as a monopartite signal which binds to the high affinity site on KPNA2/importin-alpha.DOMAIN Both the spacer region (also known as the recognition (R) domain) and C-terminal domain are required for stable binding to the DNA repair bubble (PubMed:16246722). However, both domains are dispensable for incision of DNA bubble structures (PubMed:16246722, PubMed:32522879, PubMed:32821917).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 Includes a cryptic exon found in intron 6.SIMILARITY Belongs to the XPG/RAD2 endonuclease family. XPG subfamily.CAUTION A paper describing an additional role for this protein in a base excision repair pathway that is not coupled to transcription has been retracted, because some of the experimental data were incorrect.
crossReference
databaseName UniProt
dbId 67448
description
  • recommendedName: fullName evidence="33"DNA excision repair protein ERCC-5 ecNumber evidence="18 19 21 23 24 25"3.1.-.- alternativeName: DNA repair protein complementing XP-G cells shortName: XPG alternativeName: Xeroderma pigmentosum group G-complementing protein
displayName UniProt:P28715 ERCC5
geneName
  • ERCC5
  • ERCM2
  • XPG
  • XPGC
identifier P28715
isSequenceChanged false
keyword
  • 3D-structure
  • Acetylation
  • Alternative splicing
  • Chromosome
  • Cockayne syndrome
  • Deafness
  • Disease variant
  • DNA damage
  • DNA repair
  • DNA-binding
  • Dwarfism
  • Endonuclease
  • Hydrolase
  • Magnesium
  • Metal-binding
  • Nuclease
  • Nucleus
  • Phosphoprotein
  • Proteomics identification
  • Reference proteome
  • Xeroderma pigmentosum
modified [InstanceEdit:9983091] Weiser, Joel, 2026-02-20
moleculeType Protein
name
  • ERCC5
otherIdentifier
  • 11717914_a_at
  • 11717915_s_at
  • 11748948_a_at
  • 11752129_a_at
  • 202414_PM_at
  • 202414_at
  • 2063_at
  • 2064_g_at
  • 2073
  • 233415_PM_at
  • 233415_at
  • 3499633
  • 3499634
  • 3499635
  • 3499636
  • 3499637
  • 3499638
  • 3499640
  • 3499644
  • 3499646
  • 3499647
  • 3499648
  • 3499649
  • 3499650
  • 3499653
  • 3499654
  • 3499655
  • 3499656
  • 3499657
  • 3499658
  • 3499659
  • 3499660
  • 3499661
  • 3499662
  • 3499663
  • 3499664
  • 3499665
  • 3499666
  • 3499667
  • 3499668
  • 3499669
  • 3499670
  • 3499671
  • 3499672
  • 3499673
  • 3499674
  • 3499675
  • 3499676
  • 3499677
  • 3499678
  • 3499679
  • 3499681
  • 7969935
  • A_14_P107601
  • A_14_P114951
  • A_14_P124571
  • A_23_P117225
  • A_33_P3384642
  • A_33_P3384657
  • GE59966
  • GO:0000109
  • GO:0000405
  • GO:0000724
  • GO:0000993
  • GO:0003677
  • GO:0003684
  • GO:0003690
  • GO:0003697
  • GO:0003824
  • GO:0004518
  • GO:0004519
  • GO:0004520
  • GO:0005515
  • GO:0005634
  • GO:0005654
  • GO:0005662
  • GO:0005694
  • GO:0006281
  • GO:0006283
  • GO:0006285
  • GO:0006289
  • GO:0006310
  • GO:0006974
  • GO:0008047
  • GO:0009411
  • GO:0010225
  • GO:0016787
  • GO:0016788
  • GO:0032991
  • GO:0042803
  • GO:0043066
  • GO:0044877
  • GO:0046872
  • GO:0098772
  • GO:0140097
  • HMNXSV003002070
  • HMNXSV003047568
  • HMNXSV003053245
  • Hs.291660.0.S1_3p_at
  • ILMN_1795495
  • PH_hs_0032358
  • TC13001721.hg
  • X69978_at
  • g4503600_3p_at
physicalEntity
referenceDatabase [ReferenceDatabase:2] UniProt
referenceGene
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • ERCC5_HUMAN
  • A6NGT4
  • Q5JUS4
  • Q5JUS5
  • Q7Z2V3
  • Q8IZL6
  • Q8N1B7
  • Q9HD59
  • Q9HD60
sequenceLength 1186
species [Species:48887] Homo sapiens
stId uniprot:P28715
url http://purl.uniprot.org/uniprot/P28715
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