ERCC1

Functionⁱ

Isoform 1: Non-catalytic component of a structure-specific DNA repair endonuclease responsible for the 5'-incision during DNA repair. Responsible, in conjunction with SLX4, for the first step in the repair of interstrand cross-links (ICL). Participates in the processing of anaphase bridge-generating DNA structures, which consist in incompletely processed DNA lesions arising during S or G2 phase, and can result in cytokinesis failure. Also required for homology-directed repair (HDR) of DNA double-strand breaks, in conjunction with SLX4.

Regions

Feature key	Position(s)	DescriptionActions	Graphical view	Length
DNA bindingⁱ	134 – 156	Sequence analysisAdd BLAST		23

GO - Molecular functionⁱ

damaged DNA binding
Source: UniProtKB
Inferred from direct assayⁱ
- 17720715
protein C-terminus binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 9722633
protein domain specific binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 17720715
single-stranded DNA binding
Source: UniProtKB
Inferred from direct assayⁱ
- 16076955
- 17720715
single-stranded DNA endodeoxyribonuclease activity
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
TFIID-class transcription factor complex binding Source: Ensembl

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

cell proliferation Source: Ensembl
DNA recombination
Source: MGI
Inferred from genetic interactionⁱ
- 11707424
DNA repair
Source: MGI
Inferred from mutant phenotypeⁱ
- 25538220
double-strand break repair via nonhomologous end joining
Source: BHF-UCL
Inferred from mutant phenotypeⁱ
- 14690602
embryonic organ development Source: Ensembl
global genome nucleotide-excision repair Source: Reactome
interstrand cross-link repair Source: Reactome
isotype switching Source: Ensembl
male gonad development Source: Ensembl
meiotic mismatch repair
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
mitotic recombination
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 8811092
multicellular organism aging Source: Ensembl
multicellular organism growth Source: Ensembl
negative regulation of protection from non-homologous end joining at telomere
Source: BHF-UCL
Inferred from mutant phenotypeⁱ
- 14690602
negative regulation of telomere maintenance
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 17055345
nucleotide-excision repair
Source: MGI
Inferred from direct assayⁱ
- 3290851
nucleotide-excision repair, DNA incision Source: Reactome
nucleotide-excision repair, DNA incision, 3'-to lesion
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 7657672
nucleotide-excision repair, DNA incision, 5'-to lesion
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 7657672
nucleotide-excision repair, preincision complex stabilization Source: Reactome
oogenesis Source: Ensembl
positive regulation of t-circle formation Source: BHF-UCL
post-embryonic hemopoiesis Source: Ensembl
pyrimidine dimer repair by nucleotide-excision repair Source: Ensembl
replicative cell aging Source: Ensembl
response to cadmium ion Source: Ensembl
response to immobilization stress Source: Ensembl
response to nutrient Source: Ensembl
response to oxidative stress
Source: UniProtKB
Inferred from mutant phenotypeⁱ
- 17614221
- 9256505
response to sucrose Source: Ensembl
response to X-ray Source: Ensembl
spermatogenesis Source: Ensembl
syncytium formation Source: Ensembl
t-circle formation Source: BHF-UCL
telomeric DNA-containing double minutes formation
Source: BHF-UCL
Inferred from mutant phenotypeⁱ
- 14690602
transcription-coupled nucleotide-excision repair Source: Reactome
UV-damage excision repair
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
UV protection Source: Ensembl

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	DNA-binding, Endonuclease, Hydrolase, Nuclease
Biological process	DNA damage, DNA repair

Enzyme and pathway databases

Reactomeⁱ	R-HSA-5685938 HDR through Single Strand Annealing (SSA) R-HSA-5696395 Formation of Incision Complex in GG-NER R-HSA-5696400 Dual Incision in GG-NER R-HSA-6782135 Dual incision in TC-NER R-HSA-6783310 Fanconi Anemia Pathway
SIGNORⁱ	P07992

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: DNA excision repair protein ERCC-1
Gene namesⁱ	Name:ERCC1
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 19

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000012061.15
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:3433 ERCC1
MIMⁱ	126380 gene
neXtProtⁱ	NX_P07992

Keywords - Cellular componentⁱ

Cytoplasm, Nucleus

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Cerebro-oculo-facio-skeletal syndrome 4 (COFS4)2 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionA disorder of prenatal onset characterized by microcephaly, congenital cataracts, facial dysmorphism, neurogenic arthrogryposis, growth failure and severe psychomotor retardation. COFS is considered to be part of the nucleotide-excision repair disorders spectrum that include also xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome.

Keywords - Diseaseⁱ

Cataract

Organism-specific databases

DisGeNET More...DisGeNETⁱ	2067
GeneReviewsⁱ	ERCC1
MalaCards human disease database More...MalaCardsⁱ	ERCC1
MIMⁱ	610758 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000012061
Orphanetⁱ	90322 Cockayne syndrome type 2 1466 COFS syndrome
PharmGKBⁱ	PA155

Chemistry databases

ChEMBLⁱ	CHEMBL3883316

ChEMBLⁱ

CHEMBL3883316

Polymorphism and mutation databases

BioMutaⁱ	ERCC1
DMDMⁱ	119538

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000087006	1 – 297	DNA excision repair protein ERCC-1Add BLAST		297

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	1	N-acetylmethionineCombined sources	1
Cross-linkⁱ	21	Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Cross-linkⁱ	37	Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Cross-linkⁱ	243	Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources

Keywords - PTMⁱ

Acetylation, Isopeptide bond, Ubl conjugation

Proteomic databases

EPDⁱ	P07992
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P07992
PaxDbⁱ	P07992
PeptideAtlas More...PeptideAtlasⁱ	P07992
PRIDEⁱ	P07992
ProteomicsDBⁱ	52055 52056 [P07992-2] 52057 [P07992-3]

PTM databases

iPTMnetⁱ	P07992
PhosphoSitePlusⁱ	P07992

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000012061 Expressed in 227 organ(s), highest expression level in apex of heart
CleanExⁱ	HS_ERCC1
ExpressionAtlasⁱ	P07992 baseline and differential
Genevisibleⁱ	P07992 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	CAB004390 CAB072859 CAB072860 HPA029773 HPA050182

HPAⁱ

CAB004390
CAB072859
CAB072860
HPA029773
HPA050182

Interactionⁱ

Subunit structureⁱ

Heterodimer composed of ERCC1 isoform 1 and XPF/ERRC4.3 Publications

Binary interactionsⁱ

With	Entry	#Exp.	IntAct
EGFR	P00533	21	EBI-750962,EBI-297353
ERCC4	Q92889	12	EBI-750962,EBI-2370770
MKRN3	Q13064	3	EBI-750962,EBI-2340269
MKRN3	Q6NSB6	3	EBI-750962,EBI-10195599
SLX4	Q8IY92	6	EBI-750962,EBI-2370740
TRMT2B	Q96GJ1	3	EBI-750962,EBI-10195625
UBL7	Q96S82	4	EBI-750962,EBI-348604
USHBP1	Q8N6Y0	3	EBI-750962,EBI-739895
VPS37B	Q9H9H4	3	EBI-750962,EBI-4400866
XPA	P23025	3	EBI-750962,EBI-295222

GO - Molecular functionⁱ

protein C-terminus binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 9722633
protein domain specific binding
Source: UniProtKB
Inferred from physical interactionⁱ
- 17720715
TFIID-class transcription factor complex binding Source: Ensembl

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	108379, 38 interactors
ComplexPortalⁱ	CPX-478 ERCC1-XPF endonuclease complex [P07992-1]
CORUMⁱ	P07992
Database of interacting proteins More...DIPⁱ	DIP-24235N
IntActⁱ	P07992, 19 interactors
Molecular INTeraction database More...MINTⁱ	P07992
STRINGⁱ	9606.ENSP00000013807

Structureⁱ

Secondary structure

Legend: HelixTurnBeta strandPDB Structure known for this area

Feature key	Position(s)	DescriptionActions	Length
Beta strandⁱ	101 – 103	Combined sources	3
Helixⁱ	105 – 107	Combined sources	3
Helixⁱ	111 – 115	Combined sources	5
Beta strandⁱ	121 – 123	Combined sources	3
Beta strandⁱ	127 – 133	Combined sources	7
Beta strandⁱ	136 – 142	Combined sources	7
Helixⁱ	143 – 148	Combined sources	6
Helixⁱ	152 – 160	Combined sources	9
Beta strandⁱ	163 – 172	Combined sources	10
Beta strandⁱ	175 – 177	Combined sources	3
Helixⁱ	179 – 192	Combined sources	14
Beta strandⁱ	195 – 201	Combined sources	7
Helixⁱ	202 – 213	Combined sources	12
Helixⁱ	222 – 226	Combined sources	5
Beta strandⁱ	242 – 244	Combined sources	3
Helixⁱ	247 – 257	Combined sources	11
Helixⁱ	260 – 264	Combined sources	5
Helixⁱ	268 – 272	Combined sources	5
Beta strandⁱ	274 – 276	Combined sources	3
Helixⁱ	280 – 290	Combined sources	11
Beta strandⁱ	293 – 295	Combined sources	3

Show more details

3D structure databases

ProteinModelPortalⁱ	P07992
SMRⁱ	P07992
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Miscellaneous databases

EvolutionaryTraceⁱ	P07992

EvolutionaryTraceⁱ

P07992

Family & Domainsⁱ

Region

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Regionⁱ	220 – 297	HhH2, dimerization with ERCC4Add BLAST		78

Motif

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Motifⁱ	17 – 23	Nuclear localization signalSequence analysis		7

Sequence similaritiesⁱ

Belongs to the ERCC1/RAD10/SWI10 family.Curated

Phylogenomic databases

eggNOGⁱ	KOG2841 Eukaryota COG5241 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000011275
HOGENOMⁱ	HOG000037440
HOVERGENⁱ	HBG051497
InParanoidⁱ	P07992
KEGG Orthology (KO) More...KOⁱ	K10849
OMAⁱ	LNPDYIC
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0IRO
PhylomeDBⁱ	P07992
TreeFamⁱ	TF101231

Family and domain databases

InterProⁱ	View protein in InterPro IPR004579 ERCC1/RAD10/SWI10 IPR011335 Restrct_endonuc-II-like IPR010994 RuvA_2-like
PANTHERⁱ	PTHR12749 PTHR12749, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF03834 Rad10, 1 hit
ProDomⁱ	View protein in ProDom or Entries sharing at least one domain PD013585 DNA_repair_Rad10, 1 hit
SUPFAMⁱ	SSF47781 SSF47781, 1 hit SSF52980 SSF52980, 1 hit
TIGRFAMsⁱ	TIGR00597 rad10, 1 hit

Sequences (4+)ⁱ

Sequence statusⁱ: Complete.

This entry describes 4 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 4 described isoforms and 8 potential isoforms that are computationally mapped.Show all Align All

Isoform 1 (identifier: P07992-1) [UniParc]FASTA Add to basket

Also known as: 202

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MDPGKDKEGV PQPSGPPARK KFVIPLDEDE VPPGVAKPLF RSTQSLPTVD 
        60         70         80         90        100
TSAQAAPQTY AEYAISQPLE GAGATCPTGS EPLAGETPNQ ALKPGAKSNS 
       110        120        130        140        150
IIVSPRQRGN PVLKFVRNVP WEFGDVIPDY VLGQSTCALF LSLRYHNLHP 
       160        170        180        190        200
DYIHGRLQSL GKNFALRVLL VQVDVKDPQQ ALKELAKMCI LADCTLILAW 
       210        220        230        240        250
SPEEAGRYLE TYKAYEQKPA DLLMEKLEQD FVSRVTECLT TVKSVNKTDS 
       260        270        280        290 
QTLLTTFGSL EQLIAASRED LALCPGLGPQ KARRLFDVLH EPFLKVP

Length:297

Mass (Da):32,562

Last modified:August 1, 1988 - v1

Checksum:ⁱ6FCE3615732349E5

GO

Isoform 2 (identifier: P07992-2) [UniParc]FASTA Add to basket

Also known as: 203

The sequence of this isoform differs from the canonical sequence as follows:
235-258: Missing.

« Hide

        10         20         30         40         50
MDPGKDKEGV PQPSGPPARK KFVIPLDEDE VPPGVAKPLF RSTQSLPTVD 
        60         70         80         90        100
TSAQAAPQTY AEYAISQPLE GAGATCPTGS EPLAGETPNQ ALKPGAKSNS 
       110        120        130        140        150
IIVSPRQRGN PVLKFVRNVP WEFGDVIPDY VLGQSTCALF LSLRYHNLHP 
       160        170        180        190        200
DYIHGRLQSL GKNFALRVLL VQVDVKDPQQ ALKELAKMCI LADCTLILAW 
       210        220        230        240        250
SPEEAGRYLE TYKAYEQKPA DLLMEKLEQD FVSRSLEQLI AASREDLALC 
       260        270 
PGLGPQKARR LFDVLHEPFL KVP

Note: Not functional in the nucleotide excision repair pathway. Does not interact with XPF/ERCC4.

Show »

Length:273

Mass (Da):29,993

Checksum:ⁱ04DA21E774A33524

GO

Isoform 3 (identifier: P07992-3) [UniParc]FASTA Add to basket

Also known as: 201

The sequence of this isoform differs from the canonical sequence as follows:
282-297: ARRLFDVLHEPFLKVP → VRALGKNPRSWGKERAPNKHNLRPQSFKVKKEPKTRHSGFRL

« Hide

        10         20         30         40         50
MDPGKDKEGV PQPSGPPARK KFVIPLDEDE VPPGVAKPLF RSTQSLPTVD 
        60         70         80         90        100
TSAQAAPQTY AEYAISQPLE GAGATCPTGS EPLAGETPNQ ALKPGAKSNS 
       110        120        130        140        150
IIVSPRQRGN PVLKFVRNVP WEFGDVIPDY VLGQSTCALF LSLRYHNLHP 
       160        170        180        190        200
DYIHGRLQSL GKNFALRVLL VQVDVKDPQQ ALKELAKMCI LADCTLILAW 
       210        220        230        240        250
SPEEAGRYLE TYKAYEQKPA DLLMEKLEQD FVSRVTECLT TVKSVNKTDS 
       260        270        280        290        300
QTLLTTFGSL EQLIAASRED LALCPGLGPQ KVRALGKNPR SWGKERAPNK 
       310        320 
HNLRPQSFKV KKEPKTRHSG FRL

Note: Not functional in the nucleotide excision repair pathway. Does not interact with XPF/ERCC4.

Show »

Length:323

Mass (Da):35,563

Checksum:ⁱD99BFAC9CE8E912E

GO

Isoform 4 (identifier: P07992-4) [UniParc]FASTA Add to basket

Also known as: 204

The sequence of this isoform differs from the canonical sequence as follows:
36-107: Missing.

« Hide

        10         20         30         40         50
MDPGKDKEGV PQPSGPPARK KFVIPLDEDE VPPGVRGNPV LKFVRNVPWE 
        60         70         80         90        100
FGDVIPDYVL GQSTCALFLS LRYHNLHPDY IHGRLQSLGK NFALRVLLVQ 
       110        120        130        140        150
VDVKDPQQAL KELAKMCILA DCTLILAWSP EEAGRYLETY KAYEQKPADL 
       160        170        180        190        200
LMEKLEQDFV SRVTECLTTV KSVNKTDSQT LLTTFGSLEQ LIAASREDLA 
       210        220 
LCPGLGPQKA RRLFDVLHEP FLKVP

Note: Not functional in the nucleotide excision repair pathway. Does not interact with XPF/ERCC4.

Show »

Length:225

Mass (Da):25,211

Checksum:ⁱAF78F4C26AC7DA7E

GO

Computationally mapped potential isoform sequencesⁱ

There are 8 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

K7ES46	K7ES46_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	209	Annotation score:
K7ER60	K7ER60_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	213	Annotation score:
K7ER89	K7ER89_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	234	Annotation score:
K7EP14	K7EP14_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	258	Annotation score:
K7EJW9	K7EJW9_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	112	Annotation score:
K7EMT9	K7EMT9_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	91	Annotation score:
K7EK97	K7EK97_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	141	Annotation score:
K7EJL2	K7EJL2_HUMAN	DNA excision repair protein ERCC-1 DNA excision repair protein ERCC-1	ERCC1	45	Annotation score:

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	53	A → P in BAG37398 (PubMed:14702039).Curated		1

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_032776	231	F → L in COFS4; does not alter interaction with XPF/ERCC4 or GTF2H1. 2 PublicationsCorresponds to variant dbSNP:rs121913028EnsemblClinVar.		1
Natural variantⁱVAR_019167	266	A → T1 PublicationCorresponds to variant dbSNP:rs3212977Ensembl.		1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Length
Alternative sequenceⁱVSP_053474	36 – 107	Missing in isoform 4. 1 PublicationAdd BLAST	72
Alternative sequenceⁱVSP_042727	235 – 258	Missing in isoform 2. 1 PublicationAdd BLAST	24
Alternative sequenceⁱVSP_043455	282 – 297	ARRLF…FLKVP → VRALGKNPRSWGKERAPNKH NLRPQSFKVKKEPKTRHSGF RL in isoform 3. 1 PublicationAdd BLAST	16

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	M13194 mRNA Translation: AAA52394.1 M26163 Genomic DNA Translation: AAA52395.1 M28650 mRNA Translation: AAA35810.1 AF001925 mRNA Translation: AAC16253.1 AB069681 mRNA Translation: BAB62810.1 BT019806 mRNA Translation: AAV38609.1 AF512555 Genomic DNA Translation: AAM34796.1 AK092039 mRNA Translation: BAG52472.1 AK314884 mRNA Translation: BAG37398.1 AC092309 Genomic DNA No translation available. AC138128 Genomic DNA No translation available. AC138534 Genomic DNA No translation available. AC139353 Genomic DNA No translation available. CH471126 Genomic DNA Translation: EAW57349.1 BC008930 mRNA Translation: AAH08930.1 BC052813 mRNA Translation: AAH52813.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS12662.1 [P07992-1] CCDS12663.1 [P07992-3] CCDS54279.1 [P07992-2]
PIRⁱ	A32875 A24781
NCBI Reference Sequences More...RefSeqⁱ	NP_001159521.1, NM_001166049.1 [P07992-2] NP_001974.1, NM_001983.3 [P07992-1] NP_973730.1, NM_202001.2 [P07992-3] XP_005258691.1, XM_005258634.1 [P07992-3] XP_005258692.1, XM_005258635.2 [P07992-3] XP_005258693.1, XM_005258636.4 [P07992-3] XP_011524912.1, XM_011526610.2 [P07992-3] XP_016881948.1, XM_017026459.1 [P07992-3] XP_016881949.1, XM_017026460.1 [P07992-1] XP_016881950.1, XM_017026461.1 [P07992-1] XP_016881951.1, XM_017026462.1 [P07992-1] XP_016881952.1, XM_017026463.1 [P07992-1] XP_016881953.1, XM_017026464.1 [P07992-1] XP_016881954.1, XM_017026465.1 [P07992-2] XP_016881955.1, XM_017026466.1 [P07992-2]
UniGeneⁱ	Hs.435981

Genome annotation databases

Ensemblⁱ	ENST00000013807; ENSP00000013807; ENSG00000012061 [P07992-3] ENST00000300853; ENSP00000300853; ENSG00000012061 [P07992-1] ENST00000340192; ENSP00000345203; ENSG00000012061 [P07992-2] ENST00000423698; ENSP00000394875; ENSG00000012061 [P07992-4] ENST00000589165; ENSP00000468035; ENSG00000012061 [P07992-1]
GeneIDⁱ	2067
KEGGⁱ	hsa:2067
UCSC genome browser More...UCSCⁱ	uc002pbs.3 human [P07992-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P07992	Excision repair cross-complementing rodent repair deficiency, complementation group 1 (Includes overlapping antisense sequence), isoform CRA_a		HUMAN		297	UniRef100_P07992
ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		297
Uncharacterized protein		PANPA		297
Excision repair protein (Fragment)		PANTR		251
P07992-2	ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		273	UniRef100_K7AZW1
Uncharacterized protein		PANPA		273
P07992-3	ERCC1 isoform 8		PANTR		323	UniRef100_A0A2J8PYL0
DNA excision repair protein ERCC-1 (Fragment)		HUMAN		258
ERCC1 isoform 13 (Fragment)		PANTR		258
DNA excision repair protein ERCC-1 (Fragment)		HUMAN		234
ERCC1 isoform 15 (Fragment)		PANTR		234
+2
P07992-4	ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		225	UniRef100_A0A2I3S7W6
ERCC1 isoform 2		PONAB		225
Uncharacterized protein		PANPA		225

Protein	Similar proteins		Species	Score	Length	Source
P07992	Excision repair cross-complementing rodent repair deficiency, complementation group 1 (Includes overlapping antisense sequence), isoform CRA_a		HUMAN		297	UniRef90_P07992
ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		297
Uncharacterized protein		PANPA		297
Uncharacterized protein		CHLSB		298
Uncharacterized protein		COLAP		298
+4
P07992-2	ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		273	UniRef90_K7AZW1
Uncharacterized protein		PANPA		273
Uncharacterized protein		MACMU		274
Uncharacterized protein		COLAP		274
Uncharacterized protein		RHIRO		274
+13
P07992-3	Uncharacterized protein		MACMU		323	UniRef90_A0A1D5Q468
Uncharacterized protein		MACNE		323
Uncharacterized protein		MACFA		323
ERCC1 isoform 8		PANTR		323
DNA excision repair protein ERCC-1 (Fragment)		HUMAN		258
+18
P07992-4	DNA excision repair protein ERCC-1 (Fragment)		HUMAN		209	UniRef90_K7ES46
ERCC1 isoform 12 (Fragment)		PONAB		209
ERCC1 isoform 11 (Fragment)		PANTR		209
Uncharacterized protein		MACMU		251
Uncharacterized protein		COLAP		226
+14

Protein	Similar proteins		Species	Score	Length	Source
P07992	DNA excision repair protein ERCC-1		BOVIN		294	UniRef50_P07992
Excision repair cross-complementing rodent repair deficiency, complementation group 1 (Includes overlapping antisense sequence), isoform CRA_a		HUMAN		297
ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		297
Uncharacterized protein		PANPA		297
Uncharacterized protein		CHLSB		298
+73
P07992-2	ERCC excision repair 1, endonuclease non-catalytic subunit		OTOGA		297	UniRef50_H0XZF1
ERCC excision repair 1, endonuclease non-catalytic subunit		RABIT		295
ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		225
ERCC1 isoform 2		PONAB		225
Uncharacterized protein		PANPA		225
+59
P07992-4	ERCC excision repair 1, endonuclease non-catalytic subunit		OTOGA		297	UniRef50_H0XZF1
ERCC excision repair 1, endonuclease non-catalytic subunit		RABIT		295
ERCC excision repair 1, endonuclease non-catalytic subunit		PANTR		225
ERCC1 isoform 2		PONAB		225
Uncharacterized protein		PANPA		225
+59

Cross-referencesⁱ

Web resourcesⁱ

Atlas of Genetics and Cytogenetics in Oncology and Haematology

NIEHS-SNPs

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	M13194 mRNA Translation: AAA52394.1 M26163 Genomic DNA Translation: AAA52395.1 M28650 mRNA Translation: AAA35810.1 AF001925 mRNA Translation: AAC16253.1 AB069681 mRNA Translation: BAB62810.1 BT019806 mRNA Translation: AAV38609.1 AF512555 Genomic DNA Translation: AAM34796.1 AK092039 mRNA Translation: BAG52472.1 AK314884 mRNA Translation: BAG37398.1 AC092309 Genomic DNA No translation available. AC138128 Genomic DNA No translation available. AC138534 Genomic DNA No translation available. AC139353 Genomic DNA No translation available. CH471126 Genomic DNA Translation: EAW57349.1 BC008930 mRNA Translation: AAH08930.1 BC052813 mRNA Translation: AAH52813.1
CCDSⁱ	CCDS12662.1 [P07992-1] CCDS12663.1 [P07992-3] CCDS54279.1 [P07992-2]
PIRⁱ	A32875 A24781
RefSeqⁱ	NP_001159521.1, NM_001166049.1 [P07992-2] NP_001974.1, NM_001983.3 [P07992-1] NP_973730.1, NM_202001.2 [P07992-3] XP_005258691.1, XM_005258634.1 [P07992-3] XP_005258692.1, XM_005258635.2 [P07992-3] XP_005258693.1, XM_005258636.4 [P07992-3] XP_011524912.1, XM_011526610.2 [P07992-3] XP_016881948.1, XM_017026459.1 [P07992-3] XP_016881949.1, XM_017026460.1 [P07992-1] XP_016881950.1, XM_017026461.1 [P07992-1] XP_016881951.1, XM_017026462.1 [P07992-1] XP_016881952.1, XM_017026463.1 [P07992-1] XP_016881953.1, XM_017026464.1 [P07992-1] XP_016881954.1, XM_017026465.1 [P07992-2] XP_016881955.1, XM_017026466.1 [P07992-2]
UniGeneⁱ	Hs.435981

3D structure databases

Select the link destinations: Protein Data Bank Europe More...PDBeⁱ Protein Data Bank RCSB More...RCSB PDBⁱ Protein Data Bank Japan More...PDBjⁱ Links Updated	PDB entry	Method	Resolution (Å)	Chain	Positions	PDBsum
	1Z00	NMR	-	A	220-297	[»]
	2A1I	X-ray	1.90	A	96-227	[»]
	2A1J	X-ray	2.70	B	220-296	[»]
	2JNW	NMR	-	A	96-214	[»]
	2JPD	NMR	-	A	96-219	[»]
	2MUT	NMR	-	A	220-297	[»]
ProteinModelPortalⁱ	P07992
SMRⁱ	P07992
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	108379, 38 interactors
ComplexPortalⁱ	CPX-478 ERCC1-XPF endonuclease complex [P07992-1]
CORUMⁱ	P07992
DIPⁱ	DIP-24235N
IntActⁱ	P07992, 19 interactors
MINTⁱ	P07992
STRINGⁱ	9606.ENSP00000013807

Chemistry databases

ChEMBLⁱ	CHEMBL3883316

ChEMBLⁱ

CHEMBL3883316

PTM databases

iPTMnetⁱ	P07992
PhosphoSitePlusⁱ	P07992

Polymorphism and mutation databases

BioMutaⁱ	ERCC1
DMDMⁱ	119538

Proteomic databases

EPDⁱ	P07992
MaxQBⁱ	P07992
PaxDbⁱ	P07992
PeptideAtlasⁱ	P07992
PRIDEⁱ	P07992
ProteomicsDBⁱ	52055 52056 [P07992-2] 52057 [P07992-3]

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	2067
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000013807; ENSP00000013807; ENSG00000012061 [P07992-3] ENST00000300853; ENSP00000300853; ENSG00000012061 [P07992-1] ENST00000340192; ENSP00000345203; ENSG00000012061 [P07992-2] ENST00000423698; ENSP00000394875; ENSG00000012061 [P07992-4] ENST00000589165; ENSP00000468035; ENSG00000012061 [P07992-1]
GeneIDⁱ	2067
KEGGⁱ	hsa:2067
UCSCⁱ	uc002pbs.3 human [P07992-1]

Organism-specific databases

CTDⁱ	2067
DisGeNETⁱ	2067
EuPathDBⁱ	HostDB:ENSG00000012061.15
GeneCardsⁱ	ERCC1
GeneReviewsⁱ	ERCC1
HGNCⁱ	HGNC:3433 ERCC1
HPAⁱ	CAB004390 CAB072859 CAB072860 HPA029773 HPA050182
MalaCardsⁱ	ERCC1
MIMⁱ	126380 gene 610758 phenotype
neXtProtⁱ	NX_P07992
OpenTargetsⁱ	ENSG00000012061
Orphanetⁱ	90322 Cockayne syndrome type 2 1466 COFS syndrome
PharmGKBⁱ	PA155
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG2841 Eukaryota COG5241 LUCA
GeneTreeⁱ	ENSGT00390000011275
HOGENOMⁱ	HOG000037440
HOVERGENⁱ	HBG051497
InParanoidⁱ	P07992
KOⁱ	K10849
OMAⁱ	LNPDYIC
OrthoDBⁱ	EOG091G0IRO
PhylomeDBⁱ	P07992
TreeFamⁱ	TF101231

Enzyme and pathway databases

Reactomeⁱ	R-HSA-5685938 HDR through Single Strand Annealing (SSA) R-HSA-5696395 Formation of Incision Complex in GG-NER R-HSA-5696400 Dual Incision in GG-NER R-HSA-6782135 Dual incision in TC-NER R-HSA-6783310 Fanconi Anemia Pathway
SIGNORⁱ	P07992

Miscellaneous databases

ChiTaRSⁱ	ERCC1 human
EvolutionaryTraceⁱ	P07992
GeneWikiⁱ	ERCC1
GenomeRNAiⁱ	2067
Protein Ontology More...PROⁱ	PR:P07992
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000012061 Expressed in 227 organ(s), highest expression level in apex of heart
CleanExⁱ	HS_ERCC1
ExpressionAtlasⁱ	P07992 baseline and differential
Genevisibleⁱ	P07992 HS

Family and domain databases

InterProⁱ	View protein in InterPro IPR004579 ERCC1/RAD10/SWI10 IPR011335 Restrct_endonuc-II-like IPR010994 RuvA_2-like
PANTHERⁱ	PTHR12749 PTHR12749, 1 hit
Pfamⁱ	View protein in Pfam PF03834 Rad10, 1 hit
ProDomⁱ	View protein in ProDom or Entries sharing at least one domain PD013585 DNA_repair_Rad10, 1 hit
SUPFAMⁱ	SSF47781 SSF47781, 1 hit SSF52980 SSF52980, 1 hit
TIGRFAMsⁱ	TIGR00597 rad10, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	ERCC1_HUMAN
Accessionⁱ	P07992Primary (citable) accession number: P07992 Secondary accession number(s): B2RC01 , B3KRR0, Q7Z7F5, Q96S40
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	August 1, 1988
	Last sequence update:	August 1, 1988
	Last modified:	November 7, 2018
	This is version 185 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

3D-structure, Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
Human chromosome 19
Human chromosome 19: entries, gene names and cross-references to MIM
PDB cross-references
Index of Protein Data Bank (PDB) cross-references

UniProtKB

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UniRef

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UniProtKB - P07992 (ERCC1_HUMAN)

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DNA excision repair protein ERCC-1

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Regions

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GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Nucleus

Nucleus

Other locations

Nucleus

Nucleus

Cytosol

Nucleus

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Keywords - Diseasei

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

3D structure databases

Miscellaneous databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

Motif

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

Experimental Info

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Sequence databases

3D structure databases

Protein-protein interaction databases

Chemistry databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Web resourcesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ