Ataxin 1抗体;Ataxin 1 Antibody—艾普蒂 新品

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     Western blot analysis of Ataxin 1 in 3T3 lysates using Ataxin 1 antibody.    

  
  

Ataxin-1 is a protein encoded by the *ATXN1* gene, primarily associated with spinocerebellar ataxia type 1 (SCA1), an autosomal dominant neurodegenerative disorder. In SCA1. a CAG trinucleotide repeat expansion in *ATXN1* leads to an elongated polyglutamine tract in the Ataxin-1 protein, resulting in its misfolding, aggregation, and accumulation in neuronal nuclei. This triggers neurotoxicity, particularly in cerebellar Purkinje cells and brainstem neurons, leading to progressive motor dysfunction.

Antibodies targeting Ataxin-1 are critical tools for studying its role in SCA1 pathogenesis. They enable detection of wild-type and mutant protein expression, localization, and aggregation in cellular and animal models. Commonly used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF), these antibodies help characterize Ataxin-1’s normal functions (e.g., RNA processing, transcriptional regulation) and its pathological behavior in disease. Specific epitopes, such as the polyglutamine region or distinct domains, allow differentiation between normal and expanded forms.

Research utilizing Ataxin-1 antibodies has advanced understanding of SCA1 mechanisms, including interactions with protein partners (e.g., CIC, RBM17) and modifiers of toxicity. They also support therapeutic development, such as evaluating gene silencing or aggregation inhibitors. Validation of antibody specificity remains essential, given potential cross-reactivity with homologous proteins. Overall, Ataxin-1 antibodies are indispensable for unraveling molecular pathways in SCA1 and identifying potential interventions.