KEGG   PATHWAY: hsa03420Help
Entry
hsa03420                    Pathway                                

Name
Nucleotide excision repair - Homo sapiens (human)
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
Nucleotide excision repair
hsa03420

All organismsOrtholog table
Module
DNA polymerase delta complex [PATH:hsa03420]
RPA complex [PATH:hsa03420]
RF-C complex [PATH:hsa03420]
Holo-TFIIH complex [PATH:hsa03420]
Cul4-DDB1-DDB2 complex [PATH:hsa03420]
Cul4-DDB1-CSA complex [PATH:hsa03420]
Disease
H00076  
Cockayne syndrome
H00403  
Disorders of nucleotide excision repair
H00658  
Syndromic X-linked mental retardation
H00821  
Macular degeneration
H01428  
Xeroderma pigmentosum (XP)
Other DBs
BSID: 
GO: 
Organism
Homo sapiens (human) [GN:hsa]
Gene
9978  
RBX1; ring-box 1, E3 ubiquitin protein ligase [KO:K03868]
8450  
CUL4B; cullin 4B [KO:K10609]
8451  
CUL4A; cullin 4A [KO:K10609]
1642  
DDB1; damage specific DNA binding protein 1 [KO:K10610]
1643  
DDB2; damage specific DNA binding protein 2 [KO:K10140]
7508  
XPC; xeroderma pigmentosum, complementation group C [KO:K10838]
5887  
RAD23B; RAD23 homolog B, nucleotide excision repair protein [KO:K10839]
5886  
RAD23A; RAD23 homolog A, nucleotide excision repair protein [KO:K10839]
1069  
CETN2; centrin 2 [KO:K10840]
1161  
ERCC8; excision repair cross-complementation group 8 [KO:K10570]
2074  
ERCC6; excision repair cross-complementation group 6 [KO:K10841]
ERCC6-PGBD3; ERCC6-PGBD3 readthrough [KO:K10841]
1022  
CDK7; cyclin-dependent kinase 7 [KO:K02202] [EC:2.7.11.23 2.7.11.22]
4331  
MNAT1; MNAT1, CDK activating kinase assembly factor [KO:K10842]
902  
CCNH; cyclin H [KO:K06634]
2071  
ERCC3; excision repair cross-complementation group 3 [KO:K10843] [EC:3.6.4.12]
2068  
ERCC2; excision repair cross-complementation group 2 [KO:K10844] [EC:3.6.4.12]
404672  
GTF2H5; general transcription factor IIH subunit 5 [KO:K10845]
2965  
GTF2H1; general transcription factor IIH subunit 1 [KO:K03141]
2966  
GTF2H2; general transcription factor IIH subunit 2 [KO:K03142]
730394  
GTF2H2C_2; GTF2H2 family member C, copy 2 [KO:K03142]
728340  
GTF2H2C; GTF2H2 family member C [KO:K03142]
2967  
GTF2H3; general transcription factor IIH subunit 3 [KO:K03143]
2968  
GTF2H4; general transcription factor IIH subunit 4 [KO:K03144]
2073  
ERCC5; excision repair cross-complementation group 5 [KO:K10846]
BIVM-ERCC5; BIVM-ERCC5 readthrough [KO:K10846]
7507  
XPA; xeroderma pigmentosum, complementation group A [KO:K10847]
6117  
RPA1; replication protein A1 [KO:K07466]
6118  
RPA2; replication protein A2 [KO:K10739]
6119  
RPA3; replication protein A3 [KO:K10740]
29935  
RPA4; replication protein A4 [KO:K10741]
2072  
ERCC4; excision repair cross-complementation group 4 [KO:K10848] [EC:3.1.-.-]
2067  
ERCC1; excision repair cross-complementation group 1 [KO:K10849]
5424  
POLD1; polymerase (DNA) delta 1, catalytic subunit [KO:K02327] [EC:2.7.7.7]
5425  
POLD2; polymerase (DNA) delta 2, accessory subunit [KO:K02328]
10714  
POLD3; polymerase (DNA) delta 3, accessory subunit [KO:K03504]
57804  
POLD4; polymerase (DNA) delta 4, accessory subunit [KO:K03505]
5426  
POLE; polymerase (DNA) epsilon, catalytic subunit [KO:K02324] [EC:2.7.7.7]
5427  
POLE2; polymerase (DNA) epsilon 2, accessory subunit [KO:K02325] [EC:2.7.7.7]
54107  
POLE3; polymerase (DNA) epsilon 3, accessory subunit [KO:K02326] [EC:2.7.7.7]
56655  
POLE4; polymerase (DNA) epsilon 4, accessory subunit [KO:K03506] [EC:2.7.7.7]
5111  
PCNA; proliferating cell nuclear antigen [KO:K04802]
5981  
RFC1; replication factor C subunit 1 [KO:K10754]
5984  
RFC4; replication factor C subunit 4 [KO:K10755]
5982  
RFC2; replication factor C subunit 2 [KO:K10755]
5985  
RFC5; replication factor C subunit 5 [KO:K10756]
5983  
RFC3; replication factor C subunit 3 [KO:K10756]
3978  
LIG1; ligase I, DNA, ATP-dependent [KO:K10747] [EC:6.5.1.7 6.5.1.6 6.5.1.1]
Reference
  Authors
Sugasawa K.
  Title
[Molecular mechanism of mammalian nucleotide excision repair] Japanese
  Journal
Tanpakushitsu Kakusan Koso 46:893-901 (2001)
Reference
  Authors
Nakatsu Y.
  Title
[Transcription-coupled repair and Cockayne syndrome] Japanese
  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] Japanese
  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
Neuroscience 145:1388-96 (2007)
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
Gynecol Oncol 107:S56-71 (2007)
Reference
  Authors
Sugasawa K.
  Title
[DNA repair pathways involving Cul4A ubiquitin ligases] Japanese
  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] Japanese
  Journal
Tanpakushitsu Kakusan Koso 52:1823-31 (2007)
KO pathway
 

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