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A4159-04 Rabbit Anti-ATX1 (Ataxin 1)

Specifications
References
Clone Type
Polyclonal
Host
Rabbit
Source
Human
Swiss Prot
P54253
Isotype
IgG
Grade
Affinity Purified
Applications
WB
Crossreactivity
Dr Hu Mo Rt
Gene ID
6310
Shipping Temp
Blue Ice
Storage Temp
-20°C

Spinocerebellar ataxia 1 (SCA1), an autosomal dominant neurodegenerative disorder, is characterized by slurred speech, loss of limb coordination, and gait abnormalities resulting from the degeneration of cerebellar Purkinje cells and of a subset of brainstem neurons (1). Individuals with SCA1 have a highly polymorphic CAG repeat expansion encoding a polyglutamine tract in ataxin-1 (2). Akt phosphorylates ataxin-1 at Ser776, which regulates an association with 14-3-3. This interaction increases ataxin-1 stabilization and accumulation resulting in enhanced neurodegeneration (3). In addition, HSP70 controls the effect that phosphorylation has on ataxin-1 stability (4).

Applications
Suitable for use in Western Blot. Other applications have not been tested.
Recommended Dilutions
Western Blot: 1:1000 Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. For long-term storage, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Immunogen
Synthetic peptide corresponding to human ataxin-1.
Form
Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 0.1mg/ml BSA, 50% glycerol.
Purity
Purified by Protein A and peptide affinity chromatography.
Specificity
Recognizes endogenous levels of total human ataxin-1 protein. Species Crossreactivity: mouse, rat and D. melanogaster
References
1.Zoghbi, H.Y. and Orr, H.T. (2000) Annu Rev Neurosci 23, 217-47.Orr, H.T. et al. (1993) Nat Genet 4, 221-6.Chen, H.K. et al. (2003) Cell 113, 457-68. Jorgensen, N.D. et al. (2007) J Neurochem 102, 2040-8.
USBio References
No references available
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