ENTRY ko03420 Pathway 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 PATHWAY_MAP ko03420 Nucleotide excision repair DBLINKS GO: 0006289 ORTHOLOGY K01972 E6.5.1.2, ligA, ligB; DNA ligase (NAD+) [EC:6.5.1.2] K02202 CDK7; cyclin-dependent kinase 7 [EC:2.7.11.22 2.7.11.23] K02324 POLE; DNA polymerase epsilon subunit 1 [EC:2.7.7.7] K02325 POLE2; DNA polymerase epsilon subunit 2 [EC:2.7.7.7] K02326 POLE3; DNA polymerase epsilon subunit 3 [EC:2.7.7.7] K02327 POLD1; DNA polymerase delta subunit 1 [EC:2.7.7.7] K02328 POLD2; DNA polymerase delta subunit 2 K02335 polA; DNA polymerase I [EC:2.7.7.7] 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 K03506 POLE4; DNA polymerase epsilon subunit 4 [EC:2.7.7.7] 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] K03868 RBX1, ROC1; E3 ubiquitin-protein ligase RBX1 [EC:2.3.2.32] K04802 PCNA; proliferating cell nuclear antigen K06634 CCNH; cyclin H K07466 RFA1, RPA1, rpa; replication factor A1 K10140 DDB2; DNA damage-binding protein 2 K10570 ERCC8, CKN1, CSA; DNA excision repair protein ERCC-8 K10609 CUL4; cullin 4 K10610 DDB1; DNA damage-binding protein 1 K10739 RFA2, RPA2; replication factor A2 K10740 RPA3; replication factor A3 K10741 RPA4; replication factor A4 K10747 LIG1; DNA ligase 1 [EC:6.5.1.1 6.5.1.6 6.5.1.7] 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 K10840 CETN2; centrin-2 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 PMID:11436314 AUTHORS Sugasawa K. TITLE [Molecular mechanism of mammalian nucleotide excision repair] JOURNAL Tanpakushitsu Kakusan Koso 46:893-901 (2001) REFERENCE PMID:11436316 AUTHORS Nakatsu Y. TITLE [Transcription-coupled repair and Cockayne syndrome] JOURNAL Tanpakushitsu Kakusan Koso 46:908-15 (2001) REFERENCE PMID:11436323 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 PMID:17276014 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 Neuroscience 145:1388-96 (2007) DOI:10.1016/j.neuroscience.2006.12.020 REFERENCE PMID:17884153 AUTHORS Saldivar JS, Wu X, Follen M, Gershenson D. TITLE Nucleotide excision repair pathway review I: implications in ovarian cancer and platinum sensitivity. JOURNAL Gynecol Oncol 107:S56-71 (2007) DOI:10.1016/j.ygyno.2007.07.043 REFERENCE PMID:16922398 AUTHORS Sugasawa K. TITLE [DNA repair pathways involving Cul4A ubiquitin ligases] JOURNAL Tanpakushitsu Kakusan Koso 51:1339-44 (2006) REFERENCE PMID:18018633 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) REL_PATHWAY ko03020 RNA polymerase ko04120 Ubiquitin mediated proteolysis ///