KEGG   PATHWAY: pmur03460
Entry
pmur03460                   Pathway                                
Name
Fanconi anemia pathway - Protobothrops mucrosquamatus (Taiwan habu)
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
pmur03460  Fanconi anemia pathway
pmur03460

Other DBs
GO: 0043240
Organism
Protobothrops mucrosquamatus (Taiwan habu) [GN:pmur]
Gene
107293194  ATRIP; ATR-interacting protein [KO:K10905]
107292039  ATR; serine/threonine-protein kinase ATR [KO:K06640] [EC:2.7.11.1]
107290155  FANCM; Fanconi anemia group M protein [KO:K10896]
107292578  FAAP24; Fanconi anemia core complex-associated protein 24 [KO:K10898]
107292355  CENPS; centromere protein S [KO:K11511]
107296180  CENPX; centromere protein X isoform X1 [KO:K15360]
107301471  centromere protein X [KO:K15360]
107292217  TELO2; telomere length regulation protein TEL2 homolog [KO:K11137]
107298929  transcription factor HES-1-like [KO:K06054]
107291701  HES1; transcription factor HES-1 [KO:K06054]
107286867  FAAP100; Fanconi anemia core complex-associated protein 100 [KO:K10993]
107283487  FANCA; Fanconi anemia group A protein [KO:K10888]
107290065  FANCB; Fanconi anemia group B protein [KO:K10889]
107291725  FANCC; Fanconi anemia group C protein [KO:K10890]
107302830  FANCE; Fanconi anemia group E protein [KO:K10892]
107282758  FANCF; Fanconi anemia group F protein [KO:K10893]
114923340  FANCG; Fanconi anemia group G protein [KO:K10894]
107302238  FANCL; E3 ubiquitin-protein ligase FANCL [KO:K10606] [EC:2.3.2.27]
107297887  WD repeat-containing protein 48 [KO:K15361]
107303321  WDR48; WD repeat-containing protein 48 [KO:K15361]
107287082  USP1; ubiquitin carboxyl-terminal hydrolase 1 [KO:K11832] [EC:3.4.19.12]
107289537  UBE2T; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
107286847  FANCI; Fanconi anemia group I protein [KO:K10895]
107288693  FANCD2; LOW QUALITY PROTEIN: Fanconi anemia group D2 protein [KO:K10891]
107286819  BRCA2; breast cancer type 2 susceptibility protein [KO:K08775]
107285099  PALB2; partner and localizer of BRCA2 [KO:K10897]
107300284  DNA repair protein RAD51 homolog 3 [KO:K10870]
107286222  RAD51; DNA repair protein RAD51 homolog 1 [KO:K04482]
107285925  BRCA1; breast cancer type 1 susceptibility protein [KO:K10605] [EC:2.3.2.27]
107297891  BRIP1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]
107283163  FAN1; fanconi-associated nuclease 1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
107283789  MLH1; DNA mismatch repair protein Mlh1 [KO:K08734]
107291737  PMS2; mismatch repair endonuclease PMS2 [KO:K10858]
107302397  REV1; DNA repair protein REV1 [KO:K03515] [EC:2.7.7.-]
107287169  REV3L; DNA polymerase zeta catalytic subunit isoform X1 [KO:K02350] [EC:2.7.7.7]
107289296  POLH; DNA polymerase eta [KO:K03509] [EC:2.7.7.7]
107296803  POLI; DNA polymerase iota [KO:K03510] [EC:2.7.7.7]
107291180  POLK; DNA polymerase kappa [KO:K03511] [EC:2.7.7.7]
107298595  RMI1; LOW QUALITY PROTEIN: recQ-mediated genome instability protein 1 [KO:K10990]
107288706  RMI2; recQ-mediated genome instability protein 2 [KO:K15365]
107293415  TOP3B; LOW QUALITY PROTEIN: DNA topoisomerase 3-beta-1 [KO:K03165] [EC:5.6.2.1]
107289151  TOP3A; DNA topoisomerase 3-alpha [KO:K03165] [EC:5.6.2.1]
107283943  BLM; Bloom syndrome protein [KO:K10901] [EC:5.6.2.4]
107296194  RPA1; replication protein A 70 kDa DNA-binding subunit [KO:K07466]
107288015  RPA2; replication protein A 32 kDa subunit [KO:K10739]
107283682  RPA3; replication protein A 14 kDa subunit [KO:K10740]
107297113  MUS81; crossover junction endonuclease MUS81 [KO:K08991] [EC:3.1.22.-]
107283761  EME1; crossover junction endonuclease EME1 [KO:K10882] [EC:3.1.22.-]
107293805  EME2; probable crossover junction endonuclease EME2 [KO:K10883]
107293802  ERCC4; DNA repair endonuclease XPF [KO:K10848] [EC:3.1.-.-]
107285642  ERCC1; DNA excision repair protein ERCC-1 [KO:K10849]
107282701  structure-specific endonuclease subunit slx1 [KO:K15078] [EC:3.6.1.-]
Reference
  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
  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
  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
  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
  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
  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
pmur03430  Mismatch repair
pmur03440  Homologous recombination
KO pathway
ko03460   
LinkDB

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