Rb1614-120910-WS

A synthetic peptide from c-terminal region of human Ataxin 1 (Spinocerebellar ataxia type 1 protein) conjugated to an immunogenic carrier protein was used as the antigen.

ATXN1_HUMAN
ATXN1_MOUSE
ATXN1_RAT

Spinocerebellar ataxia type 1 protein, ATXN1, ATX1, SCA1

FUNCTION: Binds RNA in vitro. May be involved in RNA metabolism. The expansion of the polyglutamine tract may alter this function.
Defects in ATXN1 are the cause of spinocerebellar ataxia type 1 (SCA1); 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.
Subcellular location: Cytoplasm. Nucleus. Note: Colocalizes with USP7 in the nucleus.
Tissue specificity: Widely expressed throughout the body.

Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

12 months after reconstitution

This item will be shipped to you at ambient temperature in a lyophilised form.

1. Banfi S, et al. Nat. Genet. 7:513-519(1994)
2. Chen Y.W, et al. J. Biol. Chem. 279:3758-3765(2004)

For research use only

100 µl

Unconjugated antibody

NZ white rabbit

Whole serum

Polyclonal

Polyclonal, whole serum

IHC, WB (confirmed by recombinant protein). A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specific for Ataxin-1.

Human, mouse, rat. Other species not yet tested.

Lyophilised

Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.

Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com

- Click to download the Data Sheet

- Click to download the Material Safety Data Sheet