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ATX1 (Ataxin 1)

ATX1 (Ataxin 1)

Cat no: A4159-04

Supplier: United States Biological
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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 degrees C for short-term only. For long-term storage, aliquot and store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees 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.
Catalogue number: A4159-04
Reactivities: Human, Mouse, Rat, Drosophila/Arthropod
Hosts: Rabbit
Applications: Western Blot
Size: 100ul
Form: Supplied as a liquid in 10mM sodium HEPES, pH 7.5, 150mM sodium chloride, 0.1mg/ml BSA, 50% glycerol.
P type: Pab
Isotype: IgG
Purity: Purified by Protein A and peptide affinity chromatography.
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.
Additional info: Recognizes endogenous levels of total human ataxin-1 protein. Species Crossreactivity: mouse, rat and D. melanogaster

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