Fanconi anaemia protein FANCD2 (IPR029448)

Short name: FANCD2

Overlapping homologous superfamilies


Family relationships



The Fanconi Anemia (FA) pathway is responsible for interstrand crosslink DNA repair [PMID: 23444773]. The name originates the recessive syndrome known as Fanconi anemia, which causes developmental problems and cancer predisposition [PMID: 11530803]. In this pathway, the FANCI-FANCD2 (ID) complex is ubiquitinated by the FA core complex and then travels to sites of damage to coordinate repair [PMID: 20603016, PMID: 20603015]. FA pathway activation seems to trigger dissociation of FANCD2 from FANCI, coinciding with FANCD2 monoubiquitination which precedes monoubiquitination of FANCI [PMID: 22753026]. This suggests a functional separation for FANCD2 from FANCI [PMID: 23658231].

Monoubiquitinated FANCD2 functions to recruit DNA repair factors FAN1 (Fanconi-associated nuclease 1) [PMID: 20603073] and SLX4 [PMID: 21464321], suggesting that chromatin-bound FANCD2Ub is a docking platform for certain DNA repair nucleases. FANCD2 has also a role in replication fork recovery [PMID: 24556218].

GO terms

Biological Process

GO:0006281 DNA repair

Molecular Function

No terms assigned in this category.

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.