AXHdomain in Ataxins and HMG containing proteins
|SMART accession number:||SM00536|
|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:||protein binding (GO:0005515), RNA binding (GO:0003723)|
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- Evolution (species in which this domain is found)
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- Structure (3D structures containing this domain)
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