KEGG   PATHWAY: xhe03460
Entry
xhe03460                    Pathway                                
Name
Fanconi anemia pathway - Xiphophorus hellerii (green swordtail)
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
xhe03460  Fanconi anemia pathway
xhe03460

Other DBs
GO: 0043240
Organism
Xiphophorus hellerii (green swordtail) [GN:xhe]
Gene
116707746  brip1; Fanconi anemia group J protein isoform X1 [KO:K15362] [EC:5.6.2.3]
116708100  rpa1; replication protein A 70 kDa DNA-binding subunit [KO:K07466]
116708645  atr; serine/threonine-protein kinase ATR [KO:K06640] [EC:2.7.11.1]
116709120  faap24; Fanconi anemia core complex-associated protein 24 [KO:K10898]
116709357  fancm; LOW QUALITY PROTEIN: Fanconi anemia group M protein [KO:K10896]
116709563  fanca; Fanconi anemia group A protein isoform X1 [KO:K10888]
116710209  fancd2; Fanconi anemia group D2 protein [KO:K10891]
116710395  [KO:K11511]
116710455  atrip; LOW QUALITY PROTEIN: ATR-interacting protein [KO:K10905]
116710471  [KO:K15363] [EC:3.1.21.- 3.1.4.1]
116711534  fan1; fanconi-associated nuclease 1 isoform X1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
116712581  polh; DNA polymerase eta isoform X1 [KO:K03509] [EC:2.7.7.7]
116713205  mlh1; DNA mismatch repair protein Mlh1 [KO:K08734]
116713285  fancl; E3 ubiquitin-protein ligase FANCL isoform X1 [KO:K10606] [EC:2.3.2.27]
116717334  rpa2; replication protein A 32 kDa subunit [KO:K10739]
116719919  slx4; structure-specific endonuclease subunit SLX4 isoform X1 [KO:K10484]
116720734  palb2; partner and localizer of BRCA2 [KO:K10897]
116721605  [KO:K06054]
116721658  wdr48; WD repeat-containing protein 48 isoform X1 [KO:K15361]
116722106  ube2t; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
116723083  rev1; DNA repair protein REV1 isoform X1 [KO:K03515] [EC:2.7.7.-]
116723089  rad51c; DNA repair protein RAD51 homolog 3 [KO:K10870]
116723150  ercc1; DNA excision repair protein ERCC-1 [KO:K10849]
116723537  fancb; Fanconi anemia group B protein isoform X1 [KO:K10889]
116726054  hes4; transcription factor HES-4 isoform X1 [KO:K06054]
116726477  usp1; ubiquitin carboxyl-terminal hydrolase 1 isoform X1 [KO:K11832] [EC:3.4.19.12]
116726480  hes1; transcription factor HES-1 [KO:K06054]
116726597  fance; Fanconi anemia group E protein isoform X1 [KO:K10892]
116726713  brca1; breast cancer type 1 susceptibility protein isoform X1 [KO:K10605] [EC:2.3.2.27]
116726865  pms2; mismatch repair endonuclease PMS2 isoform X1 [KO:K10858]
116727007  telo2; telomere length regulation protein TEL2 homolog isoform X1 [KO:K11137]
116727503  ercc4; DNA repair endonuclease XPF [KO:K10848] [EC:3.1.-.-]
116727574  eme1; crossover junction endonuclease EME1 isoform X1 [KO:K10882] [EC:3.1.22.-]
116727638  faap100; Fanconi anemia core complex-associated protein 100 [KO:K10993]
116728730  [KO:K07466]
116729150  fancg; Fanconi anemia group G protein isoform X1 [KO:K10894]
116729396  top3b; DNA topoisomerase 3-beta-1 [KO:K03165] [EC:5.6.2.1]
116729842  fancc; Fanconi anemia group C protein isoform X1 [KO:K10890]
116730056  poli; DNA polymerase iota isoform X1 [KO:K03510] [EC:2.7.7.7]
116730322  rmi1; recQ-mediated genome instability protein 1 [KO:K10990]
116730330  polk; LOW QUALITY PROTEIN: DNA polymerase kappa [KO:K03511] [EC:2.7.7.7]
116731113  rpa3; replication protein A 14 kDa subunit [KO:K10740]
116731817  [KO:K10739]
116732008  fancf; LOW QUALITY PROTEIN: Fanconi anemia group F protein [KO:K10893]
116732064  mus81; crossover junction endonuclease MUS81 [KO:K08991] [EC:3.1.22.-]
116732782  [KO:K15078] [EC:3.6.1.-]
116733201  poln; DNA polymerase nu [KO:K16618] [EC:2.7.7.7]
116733639  rad51; DNA repair protein RAD51 homolog 1 [KO:K04482]
116733850  fanci; LOW QUALITY PROTEIN: Fanconi anemia group I protein [KO:K10895]
116734608  rev3l; DNA polymerase zeta catalytic subunit [KO:K02350] [EC:2.7.7.7]
116735016  cenpx; centromere protein X [KO:K15360]
116735386  rmi2; recQ-mediated genome instability protein 2 [KO:K15365]
116735780  top3a; DNA topoisomerase 3-alpha isoform X1 [KO:K03165] [EC:5.6.2.1]
116736410  blm; Bloom syndrome protein isoform X1 [KO:K10901] [EC:5.6.2.4]
116737613  brca2; breast cancer type 2 susceptibility protein isoform X1 [KO:K08775]
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
xhe03430  Mismatch repair
xhe03440  Homologous recombination
KO pathway
ko03460   

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