KEGG   PATHWAY: phi03460
Entry
phi03460                    Pathway                                
Name
Fanconi anemia pathway - Pseudopodoces humilis (Tibetan ground-tit)
Description
The Fanconi anemia pathway is required for the efficient repair of damaged DNA, especially interstrand cross-links (ICLs). DNA ICL is directly recognized by FANCM and associated proteins, that recruit the FA core complex. The FA core complex monoubiquitinates FANCD2 and FANCI. The monoubiquitinated FANCD2/FANCI becomes an active form and interacts with a series of DNA repair proteins and facilitates downstream repair pathways. Fanconi anemia is caused by mutations in one of at least 13 FA genes and is characterized by congenital growth abnormalities, bone marrow failure and cancer predisposition.
Class
Genetic Information Processing; Replication and repair
Pathway map
phi03460  Fanconi anemia pathway
phi03460

Other DBs
GO: 0043240
Organism
Pseudopodoces humilis (Tibetan ground-tit) [GN:phi]
Gene
102099237  STRA13; centromere protein X [KO:K15360]
102099329  RMI2; recQ-mediated genome instability protein 2 [KO:K15365]
102099954  RPA1; replication protein A 70 kDa DNA-binding subunit [KO:K07466]
102099958  POLH; DNA polymerase eta isoform X1 [KO:K03509] [EC:2.7.7.7]
102100435  HES1; transcription factor HES-1 [KO:K06054]
102100830  SLX1A; structure-specific endonuclease subunit SLX1 [KO:K15078] [EC:3.6.1.-]
102100909  ERCC4; DNA repair endonuclease XPF [KO:K10848] [EC:3.1.-.-]
102100963  RMI1; recQ-mediated genome instability protein 1 [KO:K10990]
102101553  HES4; transcription factor HES-4 isoform X2 [KO:K06054]
102102143  EME1; crossover junction endonuclease EME1 isoform X2 [KO:K10882] [EC:3.1.22.-]
102102499  POLK; DNA polymerase kappa isoform X1 [KO:K03511] [EC:2.7.7.7]
102103008  TOP3B; DNA topoisomerase 3-beta-1 isoform X2 [KO:K03165] [EC:5.6.2.1]
102103272  FANCA; Fanconi anemia group A protein [KO:K10888]
102103486  ATRIP; ATR-interacting protein [KO:K10905]
102103592  RPA3; replication protein A 14 kDa subunit [KO:K10740]
102103651  REV1; DNA repair protein REV1 isoform X2 [KO:K03515] [EC:2.7.7.-]
102103993  RAD51; DNA repair protein RAD51 homolog 1 [KO:K04482]
102104602  FAAP24; Fanconi anemia core complex-associated protein 24 [KO:K10898]
102104896  FANCM; Fanconi anemia group M protein [KO:K10896]
102105388  APITD1; centromere protein S [KO:K11511]
102105453  UBE2T; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
102105527  ERCC1; DNA excision repair protein ERCC-1 [KO:K10849]
102105698  BRCA1; breast cancer type 1 susceptibility protein isoform X1 [KO:K10605] [EC:2.3.2.27]
102106120  BRIP1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]
102106966  FANCB; Fanconi anemia group B protein [KO:K10889]
102107058  RAD51C; DNA repair protein RAD51 homolog 3 [KO:K10870]
102107501  RPA2; replication protein A 32 kDa subunit [KO:K10739]
102107555  WDR48; WD repeat-containing protein 48 isoform X1 [KO:K15361]
102107829  FAN1; fanconi-associated nuclease 1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
102108207  BLM; Bloom syndrome protein [KO:K10901] [EC:5.6.2.4]
102108535  FANCE; Fanconi anemia group E protein isoform X1 [KO:K10892]
102109651  TELO2; telomere length regulation protein TEL2 homolog [KO:K11137]
102109906  ATR; serine/threonine-protein kinase ATR isoform X1 [KO:K06640] [EC:2.7.11.1]
102111731  FAAP100; Fanconi anemia-associated protein of 100 kDa [KO:K10993]
102111736  FANCF; LOW QUALITY PROTEIN: Fanconi anemia group F protein [KO:K10893]
102112338  POLN; DNA polymerase nu [KO:K16618] [EC:2.7.7.7]
102112614  PMS2; mismatch repair endonuclease PMS2 [KO:K10858]
102112724  REV3L; DNA polymerase zeta catalytic subunit [KO:K02350] [EC:2.7.7.7]
102112914  MLH1; DNA mismatch repair protein Mlh1 [KO:K08734]
102112926  PALB2; partner and localizer of BRCA2 [KO:K10897]
102113036  BRCA2; breast cancer type 2 susceptibility protein [KO:K08775]
102113328  FANCG; Fanconi anemia group G protein [KO:K10894]
102113863  FANCD2; Fanconi anemia group D2 protein [KO:K10891]
102113924  TOP3A; DNA topoisomerase 3-alpha [KO:K03165] [EC:5.6.2.1]
102114107  FANCI; Fanconi anemia group I protein isoform X1 [KO:K10895]
102114174  USP1; ubiquitin carboxyl-terminal hydrolase 1 [KO:K11832] [EC:3.4.19.12]
102114285  FANCC; Fanconi anemia group C protein [KO:K10890]
102114297  FANCL; E3 ubiquitin-protein ligase FANCL [KO:K10606] [EC:2.3.2.27]
102114485  SLX4; structure-specific endonuclease subunit SLX4 isoform X1 [KO:K10484]
Reference
PMID:18047734
  Authors
Jacquemont C, Taniguchi T
  Title
The Fanconi anemia pathway and ubiquitin.
  Journal
BMC Biochem 8 Suppl 1:S10 (2007)
DOI:10.1186/1471-2091-8-S1-S10
Reference
PMID:19805513
  Authors
Bhagwat N, Olsen AL, Wang AT, Hanada K, Stuckert P, Kanaar R, D'Andrea A, Niedernhofer LJ, McHugh PJ
  Title
XPF-ERCC1 participates in the Fanconi anemia pathway of cross-link repair.
  Journal
Mol Cell Biol 29:6427-37 (2009)
DOI:10.1128/MCB.00086-09
Reference
PMID:21701511
  Authors
Deans AJ, West SC
  Title
DNA interstrand crosslink repair and cancer.
  Journal
Nat Rev Cancer 11:467-80 (2011)
DOI:10.1038/nrc3088
Reference
PMID:20064461
  Authors
Deans AJ, West SC
  Title
FANCM connects the genome instability disorders Bloom's Syndrome and Fanconi Anemia.
  Journal
Mol Cell 36:943-53 (2009)
DOI:10.1016/j.molcel.2009.12.006
Reference
PMID:20039786
  Authors
Muniandy PA, Liu J, Majumdar A, Liu ST, Seidman MM
  Title
DNA interstrand crosslink repair in mammalian cells: step by step.
  Journal
Crit Rev Biochem Mol Biol 45:23-49 (2010)
DOI:10.3109/10409230903501819
Reference
PMID:18518821
  Authors
Ciccia A, McDonald N, West SC
  Title
Structural and functional relationships of the XPF/MUS81 family of proteins.
  Journal
Annu Rev Biochem 77:259-87 (2008)
DOI:10.1146/annurev.biochem.77.070306.102408
Related
pathway
phi03430  Mismatch repair
phi03440  Homologous recombination
KO pathway
ko03460   

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