domain in Ataxins and HMG containing proteins
SMART accession number:SM00536
Description: unknown function
Interpro abstract (IPR003652):

Spinocerebellar ataxia type 1 is late-onset neurodegenerative diseases caused by the expansion of a CAG triplet repeat in the SCA1 gene. This results in the lengthening of a polyglutamine tract in the gene product ataxin-1 producing a toxic gain of function that results in neuronal death.

The crystal structure of the AXH domain of ataxin-1 has been determined as it exhibits significant sequence similarity to the transcription factor HBP1 [ (PUBMED:12965213) ] and has been implicated in RNA binding and self-association. The AXH domain is dimeric and contains an OB-fold, a structural motif found in many oligonucleotide-binding proteins. By comparison to other proteins that contain an OB-fold, the putative RNA-binding region has been identified. In addition, there are a number of well-conserved residues that form a second ligand-binding surface, suggesting that AXH domains interact with a common yet unidentified partner [ (PUBMED:14583607) ].

GO function:RNA binding (GO:0003723), protein binding (GO:0005515)
Family alignment:
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There are 247 AXH domains in 247 proteins in SMART's nrdb database.

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