Reactome | ERCC6 [nucleoplasm]

ERCC6 [nucleoplasm]

Stable Identifier

R-HSA-54429

Type

Protein [EntityWithAccessionedSequence]

Species

Homo sapiens

Compartment

nucleoplasm

Synonyms

CSB protein, Excision repair protein ERCC-6 (Cockayne syndrome protein CSB), DNA excision repair protein ERCC-6, Cockayne syndrome protein CSB

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Locations in the PathwayBrowser

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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)
        
        ERCC6 [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)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
        
        TC-NER incision complex:5'-incised damaged DNA:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
        
        TC-NER incision complex:5'-incised damaged DNA:trimmed nascent mRNA [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Partially open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Partially open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Open bubble damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
    - Formation of TC-NER Pre-Incision Complex (Homo sapiens)
      - ERCC6 binds stalled RNA Pol II (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
      - ERCC8 (CSA) binds stalled RNA Pol II (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
      - ERCC8:DDB1:CUL4:RBX1 ubiquitinates ERCC6 and RNA Pol II (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6:ERCC8:DDB1:CUL4:RBX1 [nucleoplasm] (Homo sapiens)
        
        Hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        Ubiquitinated,hyperphosphorylated RNA Pol II:Damaged DNA template:nascent mRNA hybrid:TFIIH:ERCC6 [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
        
        TC-NER post-incision complex:dsDNA with transcription bubble [nucleoplasm] (Homo sapiens)
        
        ERCC6 [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)
        
        ERCC6 [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)
        
        ERCC6 [nucleoplasm] (Homo sapiens)
    - Recovery of RNA synthesis after TC-NER (Homo sapiens)
      - TC-NER post-incision complex:dsDNA with transcription bubble [nucleoplasm] (Homo sapiens)
        
        ERCC6 [nucleoplasm] (Homo sapiens)

External Reference Information

External Reference

UniProt:Q03468 ERCC6

Gene Names

ERCC6, CSB

Chain

chain:1-1493

Reference Genes

BioGPS Gene:2074 ERCC6

COSMIC (genes):ERCC6 ERCC6

CTD Gene:2074 ERCC6

dbSNP Gene:2074 ERCC6

ENSEMBL:ENSG00000225830 ERCC6

ENSEMBL:ENSG00000225830.16 ERCC6

KEGG Gene (Homo sapiens):2074 ERCC6

Monarch:2074 ERCC6

NCBI Gene:2074 ERCC6

OMIM:609413 ERCC6

UCSC:Q03468 ERCC6

Reference Transcript

Other Identifiers

11740066_a_at

11740067_a_at

207347_PM_at

207347_at

2074

2080_s_at

230108_PM_at

230108_at

3288690

3288692

3288694

3288696

3288698

3288699

3288701

3288703

3288705

3288706

3288708

3288709

3288710

3288711

3288713

3288714

3288717

3288718

3288719

3288720

3288723

3288724

3288725

3288726

3288727

3288729

3288731

3288733

3288737

3288740

3288745

3288757

3288758

3288759

3288760

3288761

3288762

3288763

3288766

3288769

3288770

3288771

3288773

35043_at

49088_at

57645_at

7933509

82563_at

A_23_P1292

A_32_P230192

GE532073

GE678978

GE80893

GO:0000012

GO:0000077

GO:0000166

GO:0000303

GO:0003677

GO:0003682

GO:0003824

GO:0004386

GO:0005515

GO:0005524

GO:0005634

GO:0005654

GO:0005694

GO:0005730

GO:0006281

GO:0006283

GO:0006284

GO:0006290

GO:0006310

GO:0006325

GO:0006338

GO:0006351

GO:0006355

GO:0006362

GO:0006366

GO:0006974

GO:0006979

GO:0007254

GO:0007399

GO:0008023

GO:0008094

GO:0008630

GO:0009411

GO:0009636

GO:0010165

GO:0010224

GO:0010332

GO:0012501

GO:0016787

GO:0016887

GO:0022008

GO:0023052

GO:0030154

GO:0030182

GO:0030296

GO:0031175

GO:0032784

GO:0032786

GO:0034243

GO:0035066

GO:0035264

GO:0036211

GO:0042262

GO:0043226

GO:0045494

GO:0045739

GO:0045943

GO:0045944

GO:0045945

GO:0048856

GO:0060261

GO:0090734

GO:0097680

GO:0098772

GO:0110016

GO:0140097

GO:0140657

GO:0140658

GO:1905168

GO:2001033

HMNXSV003027371

Hs.98361.0.A1_3p_at

ILMN_1786882

PH_hs_0031107

TC10001276.hg

g4557564_3p_at

Participates

as a component of

as an input of

Other forms of this molecule

Ub-ERCC6 [nucleoplasm]

Inferred To

Ercc6 [nucleoplasm] (Rattus norvegicus)

ERCC6 [nucleoplasm] (Bos taurus)

ERCC6 [nucleoplasm] (Caenorhabditis elegans)

ERCC6 [nucleoplasm] (Canis familiaris)

ERCC6 [nucleoplasm] (Danio rerio)

ERCC6 [nucleoplasm] (Gallus gallus)

ERCC6 [nucleoplasm] (Mus musculus)

ERCC6 [nucleoplasm] (Saccharomyces cerevisiae)

ERCC6 [nucleoplasm] (Schizosaccharomyces pombe)

ERCC6 [nucleoplasm] (Sus scrofa)

ercc6 [nucleoplasm] (Dictyostelium discoideum)

ercc6 [nucleoplasm] (Xenopus tropicalis)

Ghost homologue of ERCC6 [nucleoplasm] (Drosophila melanogaster)

Ghost homologue of ERCC6 [nucleoplasm] (Plasmodium falciparum)

Cross References

RefSeq

NP_000115.1, NP_001333369.1

OpenTargets

ENSG00000225830

GeneCards

Q03468

Ensembl

ENST00000355832, ENSG00000225830, ENSP00000348089

PRO

Q03468

Pharos - Targets

Q03468

Orphanet

15991

PDB

7OO3, 7OOB, 7OOP, 7OPC, 4CVO, 7OPD

Interactors (4)

Accession	#Entities	Entities	Confidence Score	Evidence (IntAct)
UniProt:P00519 ABL1	5	p-Y226,Y393-ABL1 [nucleoplasm] (R-HSA-8985251) ABL1 [cytosol] (R-HSA-375987) p-Y226,Y393-ABL1 [cytosol] (R-HSA-8942610) ABL1 [nucleoplasm] (R-HSA-5686579) p-S456-ABL1 [nucleoplasm] (R-HSA-5686576)	0.655	8
UniProt:Q13216-1 ERCC8			0.527	2
UniProt:P24928 POLR2A	7	p-S2,S7(CTD)-POLR2A [nucleoplasm] (R-HSA-6814543) p-S5,S7(CTD)-POLR2A [nucleoplasm] (R-HSA-6810226) Ub,p-S2,S5(CTD)-POLR2A [nucleoplasm] (R-HSA-6781855) p-3S-POLR2A [nucleoplasm] (R-HSA-6797701) p-S2,S5(CTD)-POLR2A [nucleoplasm] (R-HSA-113420) POLR2A [nucleoplasm] (R-HSA-63502) p-S5(CTD)-POLR2A [nucleoplasm] (R-HSA-77100)	0.499	2
UniProt:Q9HCS7 XAB2	1	XAB2 [nucleoplasm] (R-HSA-67411)	0.499	2

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