ATXN1抗体

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     Immunofluorescence analysis of NTERA-2 cells using ATXN1 mouse mAb (green). Blue: DRAQ5 fluorescent DNA dye. Red: Actin filaments have been labeled with Alexa Fluor-555 phalloidin.    

  
  

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     Flow cytometric analysis of Jurkat cells using ATXN1 mouse mAb (green) and negative control (purple).    

  
  

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     Immunohistochemical analysis of paraffin-embedded human ovarian cancer tissues (left) and lung cancer tissues (right) using ATXN1 mouse mAb with DAB staining.    

  
  

The ATXN1 antibody is a crucial tool in neuroscience and molecular biology research, primarily targeting the ataxin-1 protein encoded by the *ATXN1* gene. ATXN1 is best known for its role in spinocerebellar ataxia type 1 (SCA1), an autosomal dominant neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the *ATXN1* gene. This mutation leads to an elongated polyglutamine tract in the ataxin-1 protein, resulting in its misfolding, aggregation, and neuronal toxicity, particularly in cerebellar Purkinje cells and brainstem nuclei.

ATXN1 antibodies are widely used to detect and study the expression, localization, and post-translational modifications (e.g., phosphorylation, ubiquitination) of wild-type and mutant ataxin-1 in cellular and animal models. These antibodies aid in elucidating disease mechanisms, such as protein aggregation dynamics, interactions with co-factors (e.g., CIC, Capicua), and pathways contributing to neurodegeneration. Commercial ATXN1 antibodies are typically developed in rabbit or mouse hosts, targeting specific epitopes (e.g., N-terminal, polyQ region). Their applications span Western blotting, immunohistochemistry, immunofluorescence, and co-immunoprecipitation assays.

Research using ATXN1 antibodies has advanced therapeutic strategies, including gene silencing and small-molecule interventions targeting toxic ataxin-1 species. However, cross-reactivity with homologous proteins or polyQ sequences remains a potential limitation, necessitating careful validation. Overall, ATXN1 antibodies remain indispensable for understanding SCA1 pathology and developing targeted therapies.