KEGG   PATHWAY: ppyg03460
Entry
ppyg03460                   Pathway                                
Name
Fanconi anemia pathway - Pongo pygmaeus (Bornean orangutan)
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
ppyg03460  Fanconi anemia pathway
ppyg03460

Other DBs
GO: 0043240
Organism
Pongo pygmaeus (Bornean orangutan) [GN:ppyg]
Gene
129007356  FANCF; Fanconi anemia group F protein [KO:K10893]
129008171  CENPS; centromere protein S isoform X1 [KO:K11511]
129008408  MUS81; crossover junction endonuclease MUS81 isoform X1 [KO:K08991] [EC:3.1.22.-]
129011651  BRCA2; breast cancer type 2 susceptibility protein isoform X1 [KO:K08775]
129012960  FANCM; Fanconi anemia group M protein isoform X1 [KO:K10896]
129013691  RAD51; DNA repair protein RAD51 homolog 1 [KO:K04482]
129013729  FANCI; Fanconi anemia group I protein isoform X2 [KO:K10895]
129014212  BLM; RecQ-like DNA helicase BLM isoform X1 [KO:K10901] [EC:5.6.2.4]
129014520  FAN1; fanconi-associated nuclease 1 isoform X1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]
129015544  PALB2; partner and localizer of BRCA2 isoform X2 [KO:K10897]
129015578  ERCC4; DNA repair endonuclease XPF isoform X1 [KO:K10848] [EC:3.1.-.-]
129015841  SLX1A; structure-specific endonuclease subunit SLX1 [KO:K15078] [EC:3.6.1.-]
129015933  TELO2; telomere length regulation protein TEL2 homolog isoform X1 [KO:K11137]
129015941  EME2; probable crossover junction endonuclease EME2 isoform X2 [KO:K10883]
129016219  RMI2; RecQ-mediated genome instability protein 2 [KO:K15365]
129016231  [KO:K15365]
129016262  FANCA; Fanconi anemia group A protein isoform X5 [KO:K10888]
129016350  SLX4; structure-specific endonuclease subunit SLX4 isoform X3 [KO:K10484]
129016372  [KO:K15365]
129016649  EME1; crossover junction endonuclease EME1 isoform X1 [KO:K10882] [EC:3.1.22.-]
129017073  BRIP1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]
129017180  CENPX; centromere protein X isoform X2 [KO:K15360]
129017314  BRCA1; breast cancer type 1 susceptibility protein homolog isoform X1 [KO:K10605] [EC:2.3.2.27]
129017392  RAD51C; DNA repair protein RAD51 homolog 3 isoform X1 [KO:K10870]
129018032  TOP3A; DNA topoisomerase 3-alpha isoform X1 [KO:K03165] [EC:5.6.2.1]
129018266  RPA1; replication protein A 70 kDa DNA-binding subunit [KO:K07466]
129018372  FAAP100; Fanconi anemia core complex-associated protein 100 isoform X1 [KO:K10993]
129018757  POLI; DNA polymerase iota isoform X1 [KO:K03510] [EC:2.7.7.7]
129019557  UBE2T; ubiquitin-conjugating enzyme E2 T [KO:K13960] [EC:2.3.2.23]
129019987  ERCC1; DNA excision repair protein ERCC-1 isoform X1 [KO:K10849]
129020409  FAAP24; Fanconi anemia core complex-associated protein 24 isoform X1 [KO:K10898]
129022830  TOP3B; DNA topoisomerase 3-beta-1 isoform X1 [KO:K03165] [EC:5.6.2.1]
129023935  RPA4; replication protein A 30 kDa subunit [KO:K10741]
129024809  FANCB; LOW QUALITY PROTEIN: Fanconi anemia group B protein [KO:K10889]
129024964  FANCL; E3 ubiquitin-protein ligase FANCL isoform X1 [KO:K10606] [EC:2.3.2.27]
129030868  REV1; DNA repair protein REV1 isoform X1 [KO:K03515] [EC:2.7.7.-]
129033112  MLH1; DNA mismatch repair protein Mlh1 isoform X2 [KO:K08734]
129033188  ATR; serine/threonine-protein kinase ATR isoform X1 [KO:K06640] [EC:2.7.11.1]
129033217  FANCD2; Fanconi anemia group D2 protein isoform X1 [KO:K10891]
129033362  ATRIP; ATR-interacting protein isoform X3 [KO:K10905]
129033892  HES1; transcription factor HES-1 [KO:K06054]
129034060  WDR48; WD repeat-containing protein 48 isoform X1 [KO:K15361]
129035537  [KO:K15361]
129035766  [KO:K16618] [EC:2.7.7.7]
129036877  POLK; DNA polymerase kappa isoform X1 [KO:K03511] [EC:2.7.7.7]
129037089  [KO:K10892]
129038097  FANCE; Fanconi anemia group E protein isoform X1 [KO:K10892]
129038804  REV3L; DNA polymerase zeta catalytic subunit isoform X2 [KO:K02350] [EC:2.7.7.7]
129039473  POLH; DNA polymerase eta isoform X1 [KO:K03509] [EC:2.7.7.7]
129041031  PMS2; mismatch repair endonuclease PMS2 isoform X4 [KO:K10858]
129041227  RPA3; replication protein A 14 kDa subunit [KO:K10740]
129042402  RPA2; replication protein A 32 kDa subunit [KO:K10739]
129043782  USP1; ubiquitin carboxyl-terminal hydrolase 1 [KO:K11832] [EC:3.4.19.12]
129044085  RMI1; RecQ-mediated genome instability protein 1 [KO:K10990]
129044575  FANCC; Fanconi anemia group C protein [KO:K10890]
129044647  FANCG; Fanconi anemia group G protein isoform X1 [KO:K10894]
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
ppyg03430  Mismatch repair
ppyg03440  Homologous recombination
KO pathway
ko03460   

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