Reactome | ERCC8 [nucleoplasm]

ERCC8 [nucleoplasm]

Stable Identifier

R-HSA-52835

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

nucleoplasm

Synonyms

CSA protein, Cockayne syndrome WD-repeat protein CSA, DNA excision repair protein ERCC-8

Locations in the PathwayBrowser

Expand all

DNA Repair (Homo sapiens)

- Nucleotide Excision Repair (Homo sapiens)
  - Transcription-Coupled Nucleotide Excision Repair (TC-NER) (Homo sapiens)
    - Dual incision in TC-NER (Homo sapiens)
      - 3' incision by ERCC5 (XPG) in TC-NER (Homo sapiens)
        
        TC-NER incision complex: 5'-incised damaged DNA:trimmed nascent mRNA:(PCNA:POLD,POLE), (MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER post-incision complex:incised DNA without lesion:trimmed nascent mRNA:PCNA:(PCNA:POLD,POLE),(MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - 5' incision of damaged DNA strand by ERCC1:ERCC4 in TC-NER (Homo sapiens)
        
        TC-NER incision complex [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:RPA:ERCC5:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER incision complex:5'-incised damaged DNA:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - Binding of ERCC1:ERCC4 (ERCC1:XPF) to pre-incision complex in TC-NER (Homo sapiens)
        
        TC-NER incision complex [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:RPA:ERCC5:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:RPA:ERCC5:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - DNA polymerases delta, epsilon or kappa bind the TC-NER site (Homo sapiens)
        
        TC-NER incision complex: 5'-incised damaged DNA:trimmed nascent mRNA:(PCNA:POLD,POLE), (MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER incision complex:5'-incised damaged DNA:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - ERCC2-facilitated RNA Pol II backtracking in TC-NER (Homo sapiens)
        
        TC-NER pre-incision complex [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Partially open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Partially open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - ERCC3-facilitated RNA Pol II backtracking in TC-NER (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Partially open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Partially open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - ERCC5 and RPA bind TC-NER site (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:RPA:ERCC5:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER pre-incision complex:Open bubble damaged DNA template:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
    - Formation of TC-NER Pre-Incision Complex (Homo sapiens)
      - Assembly of the pre-incision complex in TC-NER (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1:UVSSA:USP7:XAB2 complex:XPA:TCEA1:HMGN1:EP300 [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - ERCC8 (CSA) binds stalled RNA Pol II (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1:COP9 Signalosome [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - ERCC8:DDB1:CUL4:RBX1 ubiquitinates ERCC6 and RNA Pol II (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - UVSSA:USP7 deubiquitinates ERCC6 (Homo sapiens)
        
        TC-NER pre-incision complex [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1:UVSSA:USP7:XAB2 complex:XPA:TCEA1:HMGN1:EP300 [nucleoplasm] (Homo sapiens)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:Ub:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
    - Gap-filling DNA repair synthesis and ligation in TC-NER (Homo sapiens)
      - Ligation of newly synthesized repair patch to incised DNA in TC-NER (Homo sapiens)
        
        TC-NER post-incision complex:SSB-dsDNA:trimmed nascent mRNA: (PCNA:POLD,POLE), (MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER post-incision complex:dsDNA with transcription bubble [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - Repair DNA synthesis of ~27-30 bases long patch by POLD, POLE or POLK in TC-NER (Homo sapiens)
        
        TC-NER post-incision complex:SSB-dsDNA:trimmed nascent mRNA: (PCNA:POLD,POLE), (MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
        
        TC-NER post-incision complex:incised DNA without lesion:trimmed nascent mRNA:PCNA:(PCNA:POLD,POLE),(MonoUb:K164-PCNA:POLK):RPA:RFC [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
    - Recovery of RNA synthesis after TC-NER (Homo sapiens)
      - ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)
      - TC-NER post-incision complex:dsDNA with transcription bubble [nucleoplasm] (Homo sapiens)
        
        ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        ERCC8 [nucleoplasm] (Homo sapiens)

External Reference Information

External Reference

UniProt:Q13216 ERCC8

Gene Names

ERCC8, CKN1, CSA

Chain

chain:1-396

Reference Genes

BioGPS Gene:1161 ERCC8

COSMIC (genes):ERCC8 ERCC8

CTD Gene:1161 ERCC8

dbSNP Gene:1161 ERCC8

ENSEMBL:ENSG00000049167 ERCC8

ENSEMBL_homo_sapiens_GENE:ENSG00000049167.16 ERCC8

HGNC:3439 ERCC8

KEGG Gene (Homo sapiens):1161 ERCC8

Monarch:1161 ERCC8

NCBI Gene:1161 ERCC8

OMIM:609412 ERCC8

UCSC:Q13216 ERCC8

Reference Transcript

Other Identifiers

1161

11726867_a_at

11726868_x_at

11745494_x_at

11746805_a_at

11751144_a_at

11760597_x_at

1554882_PM_at

1554882_at

1554883_PM_a_at

1554883_a_at

16996582

205162_PM_at

205162_at

2858672

2858673

2858674

2858675

2858676

2858677

2858680

2858681

2858683

2858685

2858686

2858687

2858688

2858689

2858690

2858697

2858698

2858704

2858709

2858710

2858711

2858738

2858740

2858741

2858743

2858745

2858747

2858749

39664_at

8112285

A_23_P58521

A_24_P324986

GE59050

GE681221

GE819062

GO:0000012

GO:0000109

GO:0000209

GO:0003824

GO:0004842

GO:0005515

GO:0005634

GO:0005654

GO:0006281

GO:0006283

GO:0006974

GO:0006979

GO:0009411

GO:0010165

GO:0016363

GO:0016567

GO:0016740

GO:0030163

GO:0031464

GO:0032991

GO:0036211

GO:0043161

GO:0043226

GO:0044877

GO:0045739

GO:0050896

GO:0051865

GO:0080008

GO:0090262

GO:0097680

GO:0140096

HMNXSV003036854

HMNXSV003044152

Hs2.32967.2.S1_3p_at

ILMN_1746661

ILMN_1792107

ILMN_2348146

PH_hs_0002776

TC05001394.hg

U28413_at

g4557466_3p_at

Participates

as a member of

DCAF protein [nucleoplasm] (Homo sapiens)

as a component of

ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)

Inferred To

RAD28 [nucleoplasm] (Saccharomyces cerevisiae)

ckn1 [nucleoplasm] (Schizosaccharomyces pombe)

ERCC8 [nucleoplasm] (Bos taurus)

ERCC8 [nucleoplasm] (Canis familiaris)

ERCC8 [nucleoplasm] (Gallus gallus)

ERCC8 [nucleoplasm] (Sus scrofa)

ercc8 [nucleoplasm] (Dictyostelium discoideum)

ercc8 [nucleoplasm] (Xenopus tropicalis)

Ghost homologue of ERCC8 [nucleoplasm] (Drosophila melanogaster)

Ghost homologue of ERCC8 [nucleoplasm] (Plasmodium falciparum)

Ghost homologue of ERCC8 [nucleoplasm] (Rattus norvegicus)

zgc:103550 [nucleoplasm] (Danio rerio)

csa-1 [nucleoplasm] (Caenorhabditis elegans)

Ercc8 [nucleoplasm] (Mus musculus)

Cross References

RefSeq

NP_000073.1, NP_001007235.1

OpenTargets

ENSG00000049167

HPA

ENSG00000049167-ERCC8

GeneCards

Q13216

Ensembl

ENST00000676185, ENSP00000501614, ENSP00000502154, ENSP00000494466, ENSG00000049167, ENST00000647486, ENST00000675229

PRO

Q13216

Pharos - Targets

Q13216

Orphanet

15992

PDB

8B3F, 7OOB, 4A11, 7OOP, 7OPD, 8B3D, 8B3I, 7OO3, 6FCV, 7OPC

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