KEGG PATHWAY: ko03460

PATHWAY: ko03460

Entry

ko03460 Pathway

Name

Fanconi anemia pathway

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

ko03460

Fanconi anemia pathway

Module

M00293	DNA polymerase zeta complex [PATH:ko03460]
M00295	BRCA1-associated genome surveillance complex (BASC) [PATH:ko03460]
M00413	FA core complex [PATH:ko03460]
M00414	Bloom's syndrome complex [PATH:ko03460]

Disease

H00238	Fanconi anemia
H00992	Seckel syndrome

Other DBs

BSID:

377128

GO:

0043240

Orthology

K10905	ATR interacting protein
K06640	serine/threonine-protein kinase ATR [EC:2.7.11.1]
K10896	fanconi anemia group M protein
K10898	fanconi anemia-associated protein
K11511	centromere protein S
K15360	centromere protein X
K11137	telomere length regulation protein
K06054	hairy and enhancer of split 1
K10993	fanconi anemia core complex 100 kDa subunit
K10888	fanconi anemia group A protein
K10889	fanconi anemia group B protein
K10890	fanconi anemia group C protein
K10892	fanconi anemia group E protein
K10893	fanconi anemia group F protein
K10894	fanconi anemia group G protein
K10606	E3 ubiquitin-protein ligase FANCL [EC:6.3.2.19]
K15361	WD repeat-containing protein 48
K11832	ubiquitin carboxyl-terminal hydrolase 1 [EC:3.1.2.15]
K13960	ubiquitin-conjugating enzyme E2 T [EC:6.3.2.19]
K10895	fanconi anemia group I protein
K10891	fanconi anemia group D2 protein
K08775	breast cancer 2 susceptibility protein
K10897	partner and localizer of BRCA2
K10870	RAD51-like protein 2
K04482	DNA repair protein RAD51
K10605	breast cancer type 1 susceptibility protein
K15362	fanconi anemia group J protein [EC:3.6.4.12]
K15363	fanconi-associated nuclease 1
K08734	DNA mismatch repair protein MLH1
K10858	DNA mismatch repair protein PMS2
K03515	DNA repair protein REV1 [EC:2.7.7.-]
K02350	DNA polymerase zeta [EC:2.7.7.7]
K03508	DNA polymerase zeta [EC:2.7.7.7]
K03509	DNA polymerase eta [EC:2.7.7.7]
K03510	DNA polymerase iota [EC:2.7.7.7]
K03511	DNA polymerase kappa [EC:2.7.7.7]
K16618	DNA polymerase nu [EC:2.7.7.7]
K10990	RecQ-mediated genome instability protein 1
K15364	RecQ-mediated genome instability protein 1
K15365	RecQ-mediated genome instability protein 2
K03165	DNA topoisomerase III [EC:5.99.1.2]
K10901	bloom syndrome protein [EC:3.6.4.12]
K07466	replication factor A1
K10739	replication factor A2
K10740	replication factor A3
K10741	replication factor A4
K08991	crossover junction endonuclease MUS81 [EC:3.1.22.-]
K10882	crossover junction endonuclease EME1 [EC:3.1.22.-]
K10883	crossover junction endonuclease EME2
K10848	DNA excision repair protein ERCC-4 [EC:3.1.-.-]
K10849	DNA excision repair protein ERCC-1
K15078	structure-specific endonuclease subunit SLX1 [EC:3.6.1.-]
K10484	structure-specific endonuclease subunit SLX4 (BTB/POZ domain-containing protein 12)
K15079	structure-specific endonuclease subunit SLX4

Reference

PMID:18047734

Authors

Jacquemont C, Taniguchi T

Title

The Fanconi anemia pathway and ubiquitin.

Journal

BMC Biochem 8 Suppl 1:S10 (2007)

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)

Reference

PMID:21701511

Authors

Deans AJ, West SC

Title

DNA interstrand crosslink repair and cancer.

Journal

Nat Rev Cancer 11:467-80 (2011)

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)

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)

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)

All links

Ontology (2)   
   KEGG BRITE (1)   
   GO (1)   
Pathway (7)   
   KEGG PATHWAY (2)   
   KEGG MODULE (4)   
   BioSystems (1)   
Disease (2)   
   KEGG DISEASE (2)   
Chemical reaction (10)   
   KEGG ENZYME (10)   
Gene (54)   
   KEGG ORTHOLOGY (54)   
Literature (6)   
   PubMed (6)   
All databases (81)   

Download RDF

DBGET integrated database retrieval system