Purification notesPurified from rabbit antiserum by affinity chromatography using epitope specific phosphopeptide. The antibody against non-phosphopeptide was removed by chromatography using non-phosphopeptide corresponding to the phosphorylation site.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use at an assay dependent concentration.
1/500 - 1/1000. Detects a band of approximately 87 kDa (predicted molecular weight: 87 kDa).
FunctionBinds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.
Tissue specificityWidely expressed throughout the body.
Involvement in diseaseDefects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1) [MIM:164400]; also known as olivopontocerebellar atrophy I (OPCA I or OPCA1). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
Sequence similaritiesBelongs to the ATXN1 family. Contains 1 AXH domain.
DomainThe AXH domain is required for interaction with CIC.
Post-translational modificationsPhosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract. Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract.
Cellular localizationCytoplasm. Nucleus. Colocalizes with USP7 in the nucleus.
Western blot - Ataxin 1 (phospho S776) antibody (ab63376)
All lanes : Anti-Ataxin 1 (phospho S776) antibody (ab63376) at 1/500 dilution
Lane 1 : HepG2 cell extract treated with Adriamycin (0.5 micromoles, 5hours) Lane 2 : HepG2 cell extract treated with Adriamycin (0.5 micromoles, 5hours) with immunizing phosphopeptide at 10 µg
Lysates/proteins at 30 µg per lane.
Predicted band size : 87 kDa Observed band size : 87 kDa
Immunocytochemistry/ Immunofluorescence - Ataxin 1 (phospho S776) antibody (ab63376)Image courtesy of an anonymous Abreview.
ab63376 staining Ataxin 1 (phospho S776) in human HEK cells by Immunocytochemistry/ Immunofluorescence. The cells were paraformaldehyde fixed, permeabilised in 0.01% Triton X-100 and then blocked using 30% goat serum for 30 minutes at 37°C. Samples were then incubated with primary antibody at 1/100 for 1 hour at 37°C. The secondary antibody used was a goat polyclonal conjugated to Alexa Fluor® 594 (red) used at a 1/400 dilution.