Rabbit Polyclonal ATXN1 Antibody

The ATXN1 antibody is a tool used to detect ataxin-1 (ATXN1), a protein encoded by the *ATXN1* gene. ATXN1 plays a critical role in transcriptional regulation and neuronal function, primarily localized in the nucleus. Mutations in *ATXN1*, particularly the expansion of a polyglutamine (polyQ) tract (CAG repeats), cause spinocerebellar ataxia type 1 (SCA1), a neurodegenerative disorder characterized by motor incoordination and cerebellar degeneration. The expanded polyQ stretch leads to protein aggregation, neuronal toxicity, and disrupted interactions with binding partners like CIC (capicua) transcriptional repressor. ATXN1 antibodies are essential for studying its expression, localization, and aggregation in disease models. They are used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to investigate ATXN1’s role in SCA1 pathogenesis and normal cellular processes. Some antibodies specifically target the polyQ region or flanking sequences to differentiate mutant from wild-type ATXN1. However, challenges include cross-reactivity with other polyQ-containing proteins or isoforms. Research using these antibodies has advanced understanding of SCA1 mechanisms and therapeutic strategies, such as targeting ATXN1 stability or RNA-based therapies. Validation of antibody specificity remains crucial for reliable experimental outcomes.