KEGG   PATHWAY: ptex03460
Entry
ptex03460                   Pathway                                
Name
Fanconi anemia pathway - Pseudonaja textilis (eastern brown snake)
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
ptex03460  Fanconi anemia pathway
ptex03460

Other DBs
GO: 0043240
Organism
Pseudonaja textilis (eastern brown snake) [GN:ptex]
Gene
113453118  ATRIP; ATR-interacting protein [KO:K10905]
113436869  ATR; serine/threonine-protein kinase ATR [KO:K06640] [EC:2.7.11.1]
113435159  FANCM; Fanconi anemia group M protein [KO:K10896]
113448337  Fanconi anemia core complex-associated protein 24-like [KO:K10898]
113448399  Fanconi anemia core complex-associated protein 24-like [KO:K10898]
113446247  CENPS; centromere protein S [KO:K11511]
113437076  CENPX; centromere protein X [KO:K15360]
113438704  TELO2; telomere length regulation protein TEL2 homolog isoform X1 [KO:K11137]
113450926  HES1; transcription factor HES-1 isoform X1 [KO:K06054]
113446186  HES4; transcription factor HES-4 [KO:K06054]
113437012  FAAP100; Fanconi anemia core complex-associated protein 100 [KO:K10993]
113448962  FANCA; Fanconi anemia group A protein [KO:K10888]
113445203  FANCB; Fanconi anemia group B protein [KO:K10889]
113438494  FANCC; Fanconi anemia group C protein [KO:K10890]
113449036  FANCE; Fanconi anemia group E protein [KO:K10892]
113440172  FANCF; Fanconi anemia group F protein [KO:K10893]
113449711  FANCG; Fanconi anemia group G protein [KO:K10894]
113433441  FANCL; E3 ubiquitin-protein ligase FANCL [KO:K10606] [EC:2.3.2.27]
113447971  WDR48; WD repeat-containing protein 48 [KO:K15361]
113450146  WD repeat-containing protein 48-like isoform X1 [KO:K15361]
113447948  ubiquitin carboxyl-terminal hydrolase 1-like isoform X1 [KO:K11832] [EC:3.4.19.12]
113448000  ubiquitin carboxyl-terminal hydrolase 1-like [KO:K11832] [EC:3.4.19.12]
113444314  UBE2T; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
113442029  FANCI; Fanconi anemia group I protein isoform X1 [KO:K10895]
113450315  FANCD2; Fanconi anemia group D2 protein [KO:K10891]
113438928  PALB2; partner and localizer of BRCA2 [KO:K10897]
113442595  DNA repair protein RAD51 homolog 3-like [KO:K10870]
113439168  RAD51; DNA repair protein RAD51 homolog 1 [KO:K04482]
113442541  BRIP1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]
113442335  FAN1; fanconi-associated nuclease 1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
113449511  MLH1; DNA mismatch repair protein Mlh1 [KO:K08734]
113452693  mismatch repair endonuclease PMS2-like [KO:K10858]
113452698  mismatch repair endonuclease PMS2-like [KO:K10858]
113440882  REV1; DNA repair protein REV1 isoform X1 [KO:K03515] [EC:2.7.7.-]
113455317  REV3L; DNA polymerase zeta catalytic subunit [KO:K02350] [EC:2.7.7.7]
113433155  POLH; DNA polymerase eta [KO:K03509] [EC:2.7.7.7]
113445739  POLI; DNA polymerase iota isoform X1 [KO:K03510] [EC:2.7.7.7]
113447628  POLK; DNA polymerase kappa [KO:K03511] [EC:2.7.7.7]
113438476  RMI1; recQ-mediated genome instability protein 1 [KO:K10990]
113436959  RMI2; recQ-mediated genome instability protein 2 [KO:K15365]
113446503  TOP3B; DNA topoisomerase 3-beta-1 isoform X1 [KO:K03165] [EC:5.6.2.1]
113438846  TOP3A; DNA topoisomerase 3-alpha [KO:K03165] [EC:5.6.2.1]
113442021  BLM; Bloom syndrome protein [KO:K10901] [EC:5.6.2.4]
113450011  RPA1; replication protein A 70 kDa DNA-binding subunit isoform X1 [KO:K07466]
113437447  RPA2; replication protein A 32 kDa subunit [KO:K10739]
113440120  RPA3; replication protein A 14 kDa subunit [KO:K10740]
113446654  MUS81; crossover junction endonuclease MUS81 isoform X1 [KO:K08991] [EC:3.1.22.-]
113437107  EME1; crossover junction endonuclease EME1 [KO:K10882] [EC:3.1.22.-]
113438717  EME2; probable crossover junction endonuclease EME2 isoform X1 [KO:K10883]
113438713  ERCC4; DNA repair endonuclease XPF [KO:K10848] [EC:3.1.-.-]
113450270  ERCC1; DNA excision repair protein ERCC-1 isoform X1 [KO:K10849]
113443507  structure-specific endonuclease subunit slx1-like [KO:K15078] [EC:3.6.1.-]
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
ptex03430  Mismatch repair
ptex03440  Homologous recombination
KO pathway
ko03460   
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