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Entry
Name
Nucleotide excision repair
Description
Nucleotide excision repair (NER) is a mechanism to recognize and repair bulky DNA damage caused by compounds, environmental carcinogens, and exposure to UV-light. In humans hereditary defects in the NER pathway are linked to at least three diseases: xeroderma pigmentosum (XP), Cockayne syndrome (CS), and trichothiodystrophy (TTD). The repair of damaged DNA involves at least 30 polypeptides within two different sub-pathways of NER known as transcription-coupled repair (TCR-NER) and global genome repair (GGR-NER). TCR refers to the expedited repair of lesions located in the actively transcribed strand of genes by RNA polymerase II (RNAP II). In GGR-NER the first step of damage recognition involves XPC-hHR23B complex together with XPE complex (in prokaryotes, uvrAB complex). The following steps of GGR-NER and TCR-NER are similar.
Class
Genetic Information Processing; Replication and repair
BRITE hierarchy
Pathway map
Other DBs
Orthology
K02328 POLD2; DNA polymerase delta subunit 2
K03006 RPB1, POLR2A; DNA-directed RNA polymerase II subunit RPB1 [EC:2.7.7.6 ]
K03007 RPABC5, RPB10, POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5
K03008 RPB11, POLR2J; DNA-directed RNA polymerase II subunit RPB11
K03009 RPABC4, RPB12, POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4
K03010 RPB2, POLR2B; DNA-directed RNA polymerase II subunit RPB2 [EC:2.7.7.6 ]
K03011 RPB3, POLR2C; DNA-directed RNA polymerase II subunit RPB3
K03012 RPB4, POLR2D; DNA-directed RNA polymerase II subunit RPB4
K03013 RPABC1, RPB5, POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1
K03014 RPABC2, RPB6, POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2
K03015 RPB7, POLR2G; DNA-directed RNA polymerase II subunit RPB7
K03016 RPABC3, RPB8, POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3
K03017 RPB9, POLR2I; DNA-directed RNA polymerase II subunit RPB9
K03141 TFIIH1, GTF2H1, TFB1; transcription initiation factor TFIIH subunit 1
K03142 TFIIH2, GTF2H2, SSL1; transcription initiation factor TFIIH subunit 2
K03143 TFIIH3, GTF2H3, TFB4; transcription initiation factor TFIIH subunit 3
K03144 TFIIH4, GTF2H4, TFB2; transcription initiation factor TFIIH subunit 4
K03504 POLD3; DNA polymerase delta subunit 3
K03505 POLD4; DNA polymerase delta subunit 4
K03657 uvrD, pcrA; ATP-dependent DNA helicase UvrD/PcrA [EC:5.6.2.4 ]
K03701 uvrA; excinuclease ABC subunit A
K03702 uvrB; excinuclease ABC subunit B
K03703 uvrC; excinuclease ABC subunit C
K03723 mfd; transcription-repair coupling factor (superfamily II helicase) [EC:5.6.2.4 ]
K04802 PCNA; proliferating cell nuclear antigen
K07466 RFA1, RPA1, rpa; replication factor A1
K10140 DDB2; DNA damage-binding protein 2
K10570 ERCC8, CKN1, CSA; DNA excision repair protein ERCC-8
K10610 DDB1; DNA damage-binding protein 1
K10739 RFA2, RPA2; replication factor A2
K10740 RPA3; replication factor A3
K10741 RPA4; replication factor A4
K10754 RFC1; replication factor C subunit 1
K10755 RFC2_4; replication factor C subunit 2/4
K10756 RFC3_5; replication factor C subunit 3/5
K10838 XPC; xeroderma pigmentosum group C-complementing protein
K10839 RAD23, HR23; UV excision repair protein RAD23
K10841 ERCC6, CSB, RAD26; DNA excision repair protein ERCC-6
K10842 MNAT1; CDK-activating kinase assembly factor MAT1
K10843 ERCC3, XPB; DNA excision repair protein ERCC-3 [EC:5.6.2.4 ]
K10844 ERCC2, XPD; DNA excision repair protein ERCC-2 [EC:5.6.2.3 ]
K10845 TTDA, GTF2H5, TFB5; TFIIH basal transcription factor complex TTD-A subunit
K10846 ERCC5, XPG, RAD2; DNA excision repair protein ERCC-5
K10847 XPA; DNA-repair protein complementing XP-A cells
K10848 ERCC4, XPF; DNA excision repair protein ERCC-4 [EC:3.1.-.-]
K10849 ERCC1; DNA excision repair protein ERCC-1
K21987 POLR2M, GRINL1A; DNA-directed RNA polymerase II subunit GRINL1A
K23720 UVSSA; UV-stimulated scaffold protein A
Reference
Authors
Sugasawa K.
Title
[Molecular mechanism of mammalian nucleotide excision repair]
Journal
Tanpakushitsu Kakusan Koso 46:893-901 (2001)
Reference
Authors
Nakatsu Y.
Title
[Transcription-coupled repair and Cockayne syndrome]
Journal
Tanpakushitsu Kakusan Koso 46:908-15 (2001)
Reference
Authors
Nakagawa N, Masui R, Kuramitsu S.
Title
[Structure and function of DNA repair enzyme UvrB from Thermus thermophilus HB8]
Journal
Tanpakushitsu Kakusan Koso 46:968-75 (2001)
Reference
Authors
Kraemer KH, Patronas NJ, Schiffmann R, Brooks BP, Tamura D, DiGiovanna JJ.
Title
Xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome: a complex genotype-phenotype relationship.
Journal
Reference
Authors
Saldivar JS, Wu X, Follen M, Gershenson D.
Title
Nucleotide excision repair pathway review I: implications in ovarian cancer and platinum sensitivity.
Journal
Reference
Authors
Sugasawa K.
Title
[DNA repair pathways involving Cul4A ubiquitin ligases]
Journal
Tanpakushitsu Kakusan Koso 51:1339-44 (2006)
Reference
Authors
Ito S, Ando D, Tanaka K.
Title
[Cross-talk between DNA repair and transcription: molecular mechanism and disorders]
Journal
Tanpakushitsu Kakusan Koso 52:1823-31 (2007)
Related pathway
ko04120 Ubiquitin mediated proteolysis