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Protein

General transcription and DNA repair factor IIH helicase subunit XPD

Gene

Ercc2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

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.By similarity

Catalytic activityi

ATP + H2O = ADP + phosphate.

Cofactori

Protein has several cofactor binding sites:

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi116Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi134Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi155Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi190Iron-sulfur (4Fe-4S)By similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi42 – 49ATPPROSITE-ProRule annotation8

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionDNA-binding, Helicase, Hydrolase
Biological processChromosome partition, DNA damage, DNA repair, Transcription, Transcription regulation
Ligand4Fe-4S, ATP-binding, Iron, Iron-sulfur, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-MMU-112382 Formation of RNA Pol II elongation complex
R-MMU-113418 Formation of the Early Elongation Complex
R-MMU-5696395 Formation of Incision Complex in GG-NER
R-MMU-5696400 Dual Incision in GG-NER
R-MMU-674695 RNA Polymerase II Pre-transcription Events
R-MMU-6781823 Formation of TC-NER Pre-Incision Complex
R-MMU-6782135 Dual incision in TC-NER
R-MMU-6782210 Gap-filling DNA repair synthesis and ligation in TC-NER
R-MMU-6796648 TP53 Regulates Transcription of DNA Repair Genes
R-MMU-72086 mRNA Capping
R-MMU-73762 RNA Polymerase I Transcription Initiation
R-MMU-73772 RNA Polymerase I Promoter Escape
R-MMU-73776 RNA Polymerase II Promoter Escape
R-MMU-73777 RNA Polymerase I Chain Elongation
R-MMU-73779 RNA Polymerase II Transcription Pre-Initiation And Promoter Opening
R-MMU-73863 RNA Polymerase I Transcription Termination
R-MMU-75953 RNA Polymerase II Transcription Initiation
R-MMU-75955 RNA Polymerase II Transcription Elongation
R-MMU-76042 RNA Polymerase II Transcription Initiation And Promoter Clearance
R-MMU-77075 RNA Pol II CTD phosphorylation and interaction with CE

Names & Taxonomyi

Protein namesi
Recommended name:
General transcription and DNA repair factor IIH helicase subunit XPD (EC:3.6.4.12)
Short name:
TFIIH subunit XPD
Alternative name(s):
CXPD
DNA excision repair protein ERCC-2
DNA repair protein complementing XP-D cells
Xeroderma pigmentosum group D-complementing protein
Gene namesi
Name:Ercc2
Synonyms:Xpd
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:95413 Ercc2

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cytoplasm, Cytoskeleton, Nucleus

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001019811 – 760General transcription and DNA repair factor IIH helicase subunit XPDAdd BLAST760

Post-translational modificationi

ISGylated.By similarity

Keywords - PTMi

Ubl conjugation

Proteomic databases

EPDiO08811
MaxQBiO08811
PaxDbiO08811
PRIDEiO08811

PTM databases

iPTMnetiO08811
PhosphoSitePlusiO08811

Expressioni

Gene expression databases

BgeeiENSMUSG00000030400 Expressed in 166 organ(s), highest expression level in tracheobronchial tree
ExpressionAtlasiO08811 baseline and differential
GenevisibleiO08811 MM

Interactioni

Subunit structurei

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. The interaction with GTF2H2 results in the stimulation of the 5'-->3' helicase activity. Component of the MMXD complex, which includes CIAO1, ERCC2, CIAO2B, MMS19 and SLC25A5. Interacts with CIAO1 and CIAO2B; the interaction WITH CIAO2B is direct. Interacts with ATF7IP. Interacts directly with MMS19.By similarity

GO - Molecular functioni

Protein-protein interaction databases

BioGridi199500, 1 interactor
IntActiO08811, 1 interactor
MINTiO08811
STRINGi10090.ENSMUSP00000054380

Structurei

3D structure databases

ProteinModelPortaliO08811
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini7 – 283Helicase ATP-bindingPROSITE-ProRule annotationAdd BLAST277

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni438 – 637Mediates interaction with MMS19By similarityAdd BLAST200

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi234 – 237DEAH box4

Sequence similaritiesi

Belongs to the helicase family. RAD3/XPD subfamily.Curated

Phylogenomic databases

eggNOGiKOG1131 Eukaryota
COG1199 LUCA
GeneTreeiENSGT00550000075092
HOGENOMiHOG000205390
HOVERGENiHBG051498
InParanoidiO08811
KOiK10844
OMAiKKPLRFC
OrthoDBiEOG091G0262
TreeFamiTF101232

Family and domain databases

InterProiView protein in InterPro
IPR006555 ATP-dep_Helicase_C
IPR010614 DEAD_2
IPR002464 DNA/RNA_helicase_DEAH_CS
IPR010643 HBB
IPR014013 Helic_SF1/SF2_ATP-bd_DinG/Rad3
IPR006554 Helicase-like_DEXD_c2
IPR027417 P-loop_NTPase
IPR013020 Rad3/Chl1-like
IPR001945 RAD3/XPD
PANTHERiPTHR11472:SF1 PTHR11472:SF1, 1 hit
PfamiView protein in Pfam
PF06733 DEAD_2, 1 hit
PF06777 HBB, 1 hit
PF13307 Helicase_C_2, 1 hit
PRINTSiPR00852 XRODRMPGMNTD
SMARTiView protein in SMART
SM00488 DEXDc2, 1 hit
SM00491 HELICc2, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR00604 rad3, 1 hit
PROSITEiView protein in PROSITE
PS00690 DEAH_ATP_HELICASE, 1 hit
PS51193 HELICASE_ATP_BIND_2, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

This entry has 1 described isoform and 3 potential isoforms that are computationally mapped.Show allAlign All

O08811-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MKLNVDGLLV YFPYDYIYPE QFSYMLELKR TLDAKGHGVL EMPSGTGKTV
60 70 80 90 100
SLLALIVAYQ RAYPLEVTKL IYCSRTVPEI EKVIEELRKL LSFYEQQEGE
110 120 130 140 150
KLPFLGLALS SRKNLCIHPE VTPLRFGKDV DGKCHSLTAS YVRAQYQQDA
160 170 180 190 200
SLPHCRFYEE FDIHGRQMPL PAGIYNLDDL KALGQRQGWC PYFLARYSIL
210 220 230 240 250
HANVVVYSYH YLLDPKIADL VSKELARKAV VVFDEAHNID NVCIDSMSVN
260 270 280 290 300
LTRRTLDRCQ SNLDTLQKTV LRIKETDEQR LRDEYRRLVE GLREASVARE
310 320 330 340 350
TDAHLANPVL PDEVLQEAVP GSIRTAEHFL GFLRRLLEYV KWRLRVQHVV
360 370 380 390 400
QESPPAFLSG LAQRVCIQRK PLRFCAERLR SLLHTLEIAD LADFSPLTLL
410 420 430 440 450
ANFATLVSTY AKGFTIIIEP FDDRTPTIAN PVLHFSCMDA SLAIKPVFER
460 470 480 490 500
FQSVIITSGT LSPLDIYPKI LDFHPVTMAT FTMTLARVCL CPMIIGRGND
510 520 530 540 550
QVAISSKFET REDIAVIRNY GNLLLEMSAV VPDGIVAFFT SYQYMESTVA
560 570 580 590 600
SWYEQGILEN IQRNKLLFIE TQDGAETSVA LEKYQEACEN GRGAILLSVA
610 620 630 640 650
RGKVSEGIDF VHHYGRAVIM FGVPYVYTQS RILKARLEYL RDQFQIREND
660 670 680 690 700
FLTFDAMRHA AQCVGRAIRG KTDYGLMVFA DKRFARADKR GKLPRWIQEH
710 720 730 740 750
LTDSNLNLTV DEGVQVAKYF LRQMAQPFHR EDQLGLSLLS LEQLQSEETL
760
QRIEQIAQQL
Length:760
Mass (Da):86,842
Last modified:July 27, 2011 - v2
Checksum:i02B2E116792D4E44
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E9Q6K1E9Q6K1_MOUSE
General transcription and DNA repai...
Ercc2
739Annotation score:
D3Z700D3Z700_MOUSE
General transcription and DNA repai...
Ercc2
508Annotation score:
F6YA33F6YA33_MOUSE
General transcription and DNA repai...
Ercc2
684Annotation score:

Sequence cautioni

The sequence BAB23443 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti285Y → C in BAB23443 (PubMed:16141072).Curated1
Sequence conflicti595I → V in AAB58296 (PubMed:9426063).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U97572 mRNA Translation: AAB58296.1
AK004652 mRNA Translation: BAB23443.1 Different initiation.
AK082761 mRNA Translation: BAC38607.1
CH466639 Genomic DNA Translation: EDL23140.1
CCDSiCCDS20900.1
RefSeqiNP_031975.2, NM_007949.4
UniGeneiMm.36524

Genome annotation databases

EnsembliENSMUST00000062831; ENSMUSP00000054380; ENSMUSG00000030400
GeneIDi13871
KEGGimmu:13871
UCSCiuc009flq.1 mouse

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U97572 mRNA Translation: AAB58296.1
AK004652 mRNA Translation: BAB23443.1 Different initiation.
AK082761 mRNA Translation: BAC38607.1
CH466639 Genomic DNA Translation: EDL23140.1
CCDSiCCDS20900.1
RefSeqiNP_031975.2, NM_007949.4
UniGeneiMm.36524

3D structure databases

ProteinModelPortaliO08811
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi199500, 1 interactor
IntActiO08811, 1 interactor
MINTiO08811
STRINGi10090.ENSMUSP00000054380

PTM databases

iPTMnetiO08811
PhosphoSitePlusiO08811

Proteomic databases

EPDiO08811
MaxQBiO08811
PaxDbiO08811
PRIDEiO08811

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000062831; ENSMUSP00000054380; ENSMUSG00000030400
GeneIDi13871
KEGGimmu:13871
UCSCiuc009flq.1 mouse

Organism-specific databases

CTDi2068
MGIiMGI:95413 Ercc2

Phylogenomic databases

eggNOGiKOG1131 Eukaryota
COG1199 LUCA
GeneTreeiENSGT00550000075092
HOGENOMiHOG000205390
HOVERGENiHBG051498
InParanoidiO08811
KOiK10844
OMAiKKPLRFC
OrthoDBiEOG091G0262
TreeFamiTF101232

Enzyme and pathway databases

ReactomeiR-MMU-112382 Formation of RNA Pol II elongation complex
R-MMU-113418 Formation of the Early Elongation Complex
R-MMU-5696395 Formation of Incision Complex in GG-NER
R-MMU-5696400 Dual Incision in GG-NER
R-MMU-674695 RNA Polymerase II Pre-transcription Events
R-MMU-6781823 Formation of TC-NER Pre-Incision Complex
R-MMU-6782135 Dual incision in TC-NER
R-MMU-6782210 Gap-filling DNA repair synthesis and ligation in TC-NER
R-MMU-6796648 TP53 Regulates Transcription of DNA Repair Genes
R-MMU-72086 mRNA Capping
R-MMU-73762 RNA Polymerase I Transcription Initiation
R-MMU-73772 RNA Polymerase I Promoter Escape
R-MMU-73776 RNA Polymerase II Promoter Escape
R-MMU-73777 RNA Polymerase I Chain Elongation
R-MMU-73779 RNA Polymerase II Transcription Pre-Initiation And Promoter Opening
R-MMU-73863 RNA Polymerase I Transcription Termination
R-MMU-75953 RNA Polymerase II Transcription Initiation
R-MMU-75955 RNA Polymerase II Transcription Elongation
R-MMU-76042 RNA Polymerase II Transcription Initiation And Promoter Clearance
R-MMU-77075 RNA Pol II CTD phosphorylation and interaction with CE

Miscellaneous databases

PROiPR:O08811
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000030400 Expressed in 166 organ(s), highest expression level in tracheobronchial tree
ExpressionAtlasiO08811 baseline and differential
GenevisibleiO08811 MM

Family and domain databases

InterProiView protein in InterPro
IPR006555 ATP-dep_Helicase_C
IPR010614 DEAD_2
IPR002464 DNA/RNA_helicase_DEAH_CS
IPR010643 HBB
IPR014013 Helic_SF1/SF2_ATP-bd_DinG/Rad3
IPR006554 Helicase-like_DEXD_c2
IPR027417 P-loop_NTPase
IPR013020 Rad3/Chl1-like
IPR001945 RAD3/XPD
PANTHERiPTHR11472:SF1 PTHR11472:SF1, 1 hit
PfamiView protein in Pfam
PF06733 DEAD_2, 1 hit
PF06777 HBB, 1 hit
PF13307 Helicase_C_2, 1 hit
PRINTSiPR00852 XRODRMPGMNTD
SMARTiView protein in SMART
SM00488 DEXDc2, 1 hit
SM00491 HELICc2, 1 hit
SUPFAMiSSF52540 SSF52540, 1 hit
TIGRFAMsiTIGR00604 rad3, 1 hit
PROSITEiView protein in PROSITE
PS00690 DEAH_ATP_HELICASE, 1 hit
PS51193 HELICASE_ATP_BIND_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiERCC2_MOUSE
AccessioniPrimary (citable) accession number: O08811
Secondary accession number(s): Q8C487, Q9DC01
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: July 27, 2011
Last modified: November 7, 2018
This is version 156 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
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