Anti-Ataxin-3 (ATXN3) Rabbit Polyclonal Antibody for WB, IF/ICC, ELISA - P54252

Core Product Specifications and Parameters

Biological Background: Ataxin-3 Function and Localization

• Ataxin-3 (ATXN3) is a deubiquitinating enzyme that binds long polyubiquitin chains and trims them, with weak activity on short chains (PubMed:17696782). Related references: PMID:17696782
• Ataxin-3 is involved in the degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP, where it restricts ubiquitin chain extension on STUB1/CHIP substrates (By similarity).
• Ataxin-3 acts as a histone-binding protein and represses transcription by interacting with key transcription regulators (PubMed:12297501). Related references: PMID:12297501
• Ataxin-3 negatively regulates mTORC1 signaling in response to amino acid deprivation by deubiquitinating RHEB (PubMed:33157014). Related references: PMID:33157014
• Ataxin-3 regulates autophagy by deubiquitinating BECN1 at Lys-402, leading to BECN1 stabilization (PubMed:28445460). Related references: PMID:28445460
• Ataxin-3 localizes to the nucleus matrix, nucleoplasm, and lysosome membrane.
• Ataxin-3 is ubiquitously expressed across tissues.
• Mutations in ATXN3, characterized by CAG triplet repeat expansions, cause Machado-Joseph disease (spinocerebellar ataxia type 3), a progressive neurodegenerative disorder.

Experimental Guidance and Technical Tips

• The immunogen covers amino acids 1-361 of human Ataxin-3 (NP_004984.2), representing nearly the full-length protein. For optimal results in WB, consider using whole-cell lysates from human or mouse cell lines known to express ATXN3, such as neuronal or muscle cells.
• For IF/ICC, validate the antibody in cells expressing endogenous ATXN3, as subcellular localization may vary (nuclear matrix, nucleoplasm, lysosomes).
• In ELISA applications, use recombinant Ataxin-3 protein as a positive control to establish assay sensitivity.
• Ensure adequate negative controls (e.g., ATXN3 knockout cell lines or siRNA-mediated knockdown) to verify specificity.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio specializes in providing a comprehensive range of IVD raw materials for diagnostic manufacturers, including validated antibody pairs, optimized monoclonal/polyclonal antibodies, and bulk ancillary reagents. Our Ataxin-3 antibody supports research in neurodegeneration, protein quality control, and autophagy, and we assist in sourcing rare target raw materials to accelerate your diagnostic development from concept to clinic.