Aprataxin (IPR026963)

Short name: Aprataxin

Overlapping homologous superfamilies

Family relationships



Aprataxin is a DNA-binding protein involved in single-strand and double-strand DNA break repair, and base excision repair [PMID: 15044383, PMID: 16547001, PMID: 17276982]. In yeast the aprataxin orthologue is known as Hnt3 (Hit family protein 3) [PMID: 21984208]. Aprataxin is a member of the histidine triad (HIT) superfamily of nucleotide hydrolases and transferases, named for their characteristic HxHxHxx catalytic motif, where x is a hydrophobic amino acid [PMID: 11586299]. It also contains an N-terminal forkhead associated (FHA) domain, which is thought to mediate interactions with the ligase cofactors XRCC1 and XRCC4, and a C-terminal zinc finger domain implicated in DNA binding [PMID: 15555565, PMID: 15380105].

Aprataxin repairs adenylated RNA-DNA junctions [PMID: 24362567]. It resolves abortive DNA ligation intermediates formed either at base excision sites, or when DNA ligases attempt to repair non-ligatable breaks induced by reactive oxygen species. It catalyses the release of adenylate groups covalently linked to 5'-phosphate termini, resulting in the production of 5'-phosphate termini that can be efficiently rejoined [PMID: 16964241]. Mutations in the aprataxin gene cause the neurological disorder ataxia oculomotor apraxia-1 (AOA1).

GO terms

Biological Process

GO:0006281 DNA repair

Molecular Function

GO:0033699 DNA 5'-adenosine monophosphate hydrolase activity
GO:0003677 DNA binding

Cellular Component

No terms assigned in this category.

Contributing signatures

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