KEGG PATHWAY: tge03460

KEGG

PATHWAY: tge03460

Entry

tge03460                    Pathway

Name

Fanconi anemia pathway - Theropithecus gelada (gelada)

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

Fanconi anemia pathway

Other DBs

GO:

Organism

Theropithecus gelada (gelada) [GN:tge]

Gene

FANCL; E3 ubiquitin-protein ligase FANCL isoform X1 [KO:K10606] [EC:2.3.2.27]

REV1; DNA repair protein REV1 isoform X1 [KO:K03515] [EC:2.7.7.-]

MUS81; crossover junction endonuclease MUS81 isoform X1 [KO:K08991] [EC:3.1.22.-]

FANCF; Fanconi anemia group F protein [KO:K10893]

RPA2; replication protein A 32 kDa subunit isoform X1 [KO:K10739]

FANCG; Fanconi anemia group G protein [KO:K10894]

FANCC; Fanconi anemia group C protein isoform X1 [KO:K10890]

RMI1; recQ-mediated genome instability protein 1 [KO:K10990]

CENPX; centromere protein X isoform X1 [KO:K15360]

TOP3A; DNA topoisomerase 3-alpha isoform X1 [KO:K03165] [EC:5.6.2.1]

BRIP1; Fanconi anemia group J protein [KO:K15362] [EC:5.6.2.3]

RPA1; replication protein A 70 kDa DNA-binding subunit isoform X1 [KO:K07466]

FAAP100; Fanconi anemia core complex-associated protein 100 isoform X1 [KO:K10993]

BRCA1; breast cancer type 1 susceptibility protein isoform X1 [KO:K10605] [EC:2.3.2.27]

RAD51C; DNA repair protein RAD51 homolog 3 [KO:K10870]

EME1; crossover junction endonuclease EME1 isoform X1 [KO:K10882] [EC:3.1.22.-]

BRCA2; breast cancer type 2 susceptibility protein [KO:K08775]

POLI; DNA polymerase iota isoform X1 [KO:K03510] [EC:2.7.7.7]

FAAP24; Fanconi anemia core complex-associated protein 24 isoform X1 [KO:K10898]

ERCC1; DNA excision repair protein ERCC-1 isoform X1 [KO:K10849]

EME2; probable crossover junction endonuclease EME2 [KO:K10883]

FANCA; Fanconi anemia group A protein isoform X1 [KO:K10888]

SLX4; LOW QUALITY PROTEIN: structure-specific endonuclease subunit SLX4 [KO:K10484]

TELO2; telomere length regulation protein TEL2 homolog [KO:K11137]

RMI2; recQ-mediated genome instability protein 2 [KO:K15365]

PALB2; partner and localizer of BRCA2 isoform X1 [KO:K10897]

SLX1A; structure-specific endonuclease subunit SLX1 isoform X1 [KO:K15078] [EC:3.6.1.-]

ERCC4; DNA repair endonuclease XPF [KO:K10848] [EC:3.1.-.-]

RPA4; replication protein A 30 kDa subunit [KO:K10741]

FANCB; Fanconi anemia group B protein [KO:K10889]

USP1; ubiquitin carboxyl-terminal hydrolase 1 [KO:K11832] [EC:3.4.19.12]

WDR48; WD repeat-containing protein 48 isoform X1 [KO:K15361]

ATR; serine/threonine-protein kinase ATR isoform X1 [KO:K06640] [EC:2.7.11.1]

FANCD2; Fanconi anemia group D2 protein isoform X1 [KO:K10891]

HES1; transcription factor HES-1 [KO:K06054]

ATRIP; ATR-interacting protein isoform X1 [KO:K10905]

MLH1; DNA mismatch repair protein Mlh1 [KO:K08734]

RPA3; replication protein A 14 kDa subunit [KO:K10740]

PMS2; mismatch repair endonuclease PMS2 isoform X1 [KO:K10858]

REV3L; DNA polymerase zeta catalytic subunit isoform X1 [KO:K02350] [EC:2.7.7.7]

POLH; DNA polymerase eta isoform X1 [KO:K03509] [EC:2.7.7.7]

FANCE; Fanconi anemia group E protein isoform X1 [KO:K10892]

[KO:K16618] [EC:2.7.7.7]

POLK; DNA polymerase kappa [KO:K03511] [EC:2.7.7.7]

RAD51; DNA repair protein RAD51 homolog 1 isoform X1 [KO:K04482]

FAN1; fanconi-associated nuclease 1 [KO:K15363] [EC:3.1.21.- 3.1.4.1]

UBE2T; ubiquitin-conjugating enzyme E2 T isoform X1 [KO:K13960] [EC:2.3.2.23]

FANCI; Fanconi anemia group I protein isoform X1 [KO:K10895]

FANCM; Fanconi anemia group M protein isoform X1 [KO:K10896]

BLM; Bloom syndrome protein isoform X1 [KO:K10901] [EC:5.6.2.4]

TOP3B; DNA topoisomerase 3-beta-1 isoform X1 [KO:K03165] [EC:5.6.2.1]

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

Mismatch repair

Homologous recombination

KO pathway

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