Cell Signaling Technology

Product Pathways - Neuroscience

Ataxin-1 Antibody #2177

Applications Reactivity Sensitivity MW (kDa) Source
W H M R Dm Endogenous 105 Rabbit

Applications Key:  W=Western Blotting
Reactivity Key:  H=Human  M=Mouse  R=Rat  Dm=D. melanogaster
Species cross-reactivity is determined by western blot. Species enclosed in parentheses are predicted to react based on 100% sequence homology.

Protocols

Specificity / Sensitivity

Ataxin-1 Antibody detects endogenous levels of total ataxin-1 protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to human ataxin-1. Antibodies are purified by protein A and peptide affinity chromatography.

Western Blotting

Western Blotting

Western blot analysis of extracts from rat and mouse brain using Ataxin-1 Antibody.

Background

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).

  1. Zoghbi, H.Y. and Orr, H.T. (2000) Annu Rev Neurosci 23, 217-47.
  2. Orr, H.T. et al. (1993) Nat Genet 4, 221-6.
  3. Chen, H.K. et al. (2003) Cell 113, 457-68.
  4. Jorgensen, N.D. et al. (2007) J Neurochem 102, 2040-8.

Application References

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Companion Products

For Research Use Only. Not For Use In Diagnostic Procedures.

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