**Background of ATXN3 Antibodies**
ATXN3 (ataxin-3) antibodies are essential tools for studying the ATXN3 protein, encoded by the *ATXN3* gene, which is linked to Machado-Joseph disease (MJD), also termed spinocerebellar ataxia type 3 (SCA3). This neurodegenerative disorder arises from a CAG trinucleotide repeat expansion in *ATXN3*, leading to an extended polyglutamine tract in the mutant protein. Wild-type ataxin-3 is a deubiquitinating enzyme involved in protein quality control, modulating the ubiquitin-proteasome system, and regulating cellular processes like transcription and endoplasmic reticulum stress.
ATXN3 antibodies enable the detection and characterization of both normal and pathogenic forms of the protein. They are widely used in research to investigate SCA3 pathogenesis, particularly how polyglutamine-expanded ataxin-3 forms toxic aggregates, disrupts proteostasis, and induces neuronal dysfunction. These antibodies support techniques such as Western blotting, immunohistochemistry, and immunofluorescence to analyze protein expression, localization, and aggregation patterns in cellular and animal models. Additionally, they aid in studying ataxin-3’s interactions with other proteins (e.g., ubiquitin chains, chaperones) and its role in DNA repair or transcriptional regulation.
The development of specific ATXN3 antibodies has advanced therapeutic research, including strategies to reduce toxic aggregates or enhance protein clearance. Their utility extends to diagnostic applications, such as confirming *ATXN3* mutations in clinical samples. By elucidating molecular mechanisms in SCA3. ATXN3 antibodies remain critical for understanding polyglutamine disorders and developing targeted therapies.