KEGG   PATHWAY: els03460
Entry
els03460                    Pathway                                
Name
Fanconi anemia pathway - Esox lucius (northern pike)
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
els03460  Fanconi anemia pathway
els03460

Other DBs
GO: 0043240
Organism
Esox lucius (northern pike) [GN:els]
Gene
105005798  mlh1; DNA mismatch repair protein Mlh1 isoform X1 [KO:K08734]
105005808  faap100; Fanconi anemia core complex-associated protein 100 isoform X1 [KO:K10993]
105006565  rpa2; replication protein A 32 kDa subunit [KO:K10739]
105007197  eme1; crossover junction endonuclease EME1 [KO:K10882] [EC:3.1.22.-]
105007503  brca2; breast cancer type 2 susceptibility protein isoform X1 [KO:K08775]
105009514  brip1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]
105010668  polh; DNA polymerase eta isoform X1 [KO:K03509] [EC:2.7.7.7]
105011104  rad51c; DNA repair protein RAD51 homolog 3 [KO:K10870]
105011317  hes1; transcription factor HES-1 [KO:K06054]
105011905  usp1; ubiquitin carboxyl-terminal hydrolase 1 [KO:K11832] [EC:3.4.19.12]
105012110  slx4; structure-specific endonuclease subunit SLX4 isoform X1 [KO:K10484]
105012379  rpa3; replication protein A 14 kDa subunit [KO:K10740]
105013044  cenpx; centromere protein X [KO:K15360]
105013510  atrip; ATR-interacting protein isoform X1 [KO:K10905]
105013779  hes4; transcription factor HES-4 isoform X1 [KO:K06054]
105014108  fancc; Fanconi anemia group C protein isoform X1 [KO:K10890]
105014328  polk; DNA polymerase kappa [KO:K03511] [EC:2.7.7.7]
105014451  rmi1; recQ-mediated genome instability protein 1 [KO:K10990]
105014540  top3b; DNA topoisomerase 3-beta-1 [KO:K03165] [EC:5.6.2.1]
105016002  fancm; Fanconi anemia group M protein isoform X1 [KO:K10896]
105016450  fancb; Fanconi anemia group B protein isoform X1 [KO:K10889]
105016617  rev1; DNA repair protein REV1 isoform X1 [KO:K03515] [EC:2.7.7.-]
105017169  fancd2; Fanconi anemia group D2 protein isoform X2 [KO:K10891]
105017607  rad51; DNA repair protein RAD51 homolog 1 [KO:K04482]
105017891  rev3l; DNA polymerase zeta catalytic subunit [KO:K02350] [EC:2.7.7.7]
105018093  faap24; Fanconi anemia core complex-associated protein 24 [KO:K10898]
105018191  fancf; Fanconi anemia group F protein [KO:K10893]
105018353  fan1; fanconi-associated nuclease 1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
105018415  fanci; Fanconi anemia group I protein isoform X1 [KO:K10895]
105018563  fanca; Fanconi anemia group A protein isoform X1 [KO:K10888]
105019805  atr; serine/threonine-protein kinase ATR isoform X1 [KO:K06640] [EC:2.7.11.1]
105020573  poln; LOW QUALITY PROTEIN: DNA polymerase nu [KO:K16618] [EC:2.7.7.7]
105020774  [KO:K15078] [EC:3.6.1.-]
105021267  telo2; telomere length regulation protein TEL2 homolog isoform X1 [KO:K11137]
105022935  rpa1; replication protein A 70 kDa DNA-binding subunit [KO:K07466]
105022936  [KO:K15361]
105023847  fance; Fanconi anemia group E protein [KO:K10892]
105025426  [KO:K11511]
105025567  fancg; Fanconi anemia group G protein isoform X3 [KO:K10894]
105025675  blm; Bloom syndrome protein isoform X1 [KO:K10901] [EC:5.6.2.4]
105027482  palb2; partner and localizer of BRCA2 [KO:K10897]
105028498  [KO:K07466]
105029477  mus81; crossover junction endonuclease MUS81 isoform X1 [KO:K08991] [EC:3.1.22.-]
105029812  ercc1; DNA excision repair protein ERCC-1 [KO:K10849]
105030345  fancl; E3 ubiquitin-protein ligase FANCL isoform X1 [KO:K10606] [EC:2.3.2.27]
105030365  [KO:K06054]
105030479  top3a; DNA topoisomerase 3-alpha [KO:K03165] [EC:5.6.2.1]
105030602  brca1; breast cancer type 1 susceptibility protein isoform X1 [KO:K10605] [EC:2.3.2.27]
105030615  [KO:K15361]
105030755  ube2t; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
105031067  ercc4; DNA repair endonuclease XPF isoform X1 [KO:K10848] [EC:3.1.-.-]
105031449  pms2; mismatch repair endonuclease PMS2 [KO:K10858]
114834888  [KO:K16618] [EC:2.7.7.7]
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
els03430  Mismatch repair
els03440  Homologous recombination
KO pathway
ko03460   

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