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

Core Product Specifications and Parameters

Biological Background: Ataxin-3 Function and Localization

• Ataxin-3 (ATXN3) is a deubiquitinating enzyme (DUB) that plays a critical role in protein homeostasis, transcription regulation, cytoskeleton organization, myogenesis, and the degradation of misfolded chaperone substrates. Related references: PMID:12297501, PMID:16118278, PMID:17696782
• ATXN3 specifically binds and trims long polyubiquitin chains but exhibits weak or no activity against chains of four or fewer ubiquitins. Related references: PMID:17696782
• The protein negatively regulates mTORC1 signaling during amino acid deprivation by deubiquitinating RHEB, leading to RHEB inactivation. Related references: PMID:33157014
• ATXN3 stabilizes BECN1 via deubiquitination at Lys-402, thereby regulating autophagy. Related references: PMID:28445460
• It acts as a histone-binding protein and interacts with transcription regulators, serving as a transcriptional repressor. Related references: PMID:12297501
• Subcellularly, ATXN3 localizes to the nucleus matrix, nucleus, and lysosome membrane.
• ATXN3 is ubiquitously expressed in tissues and is associated with Machado-Joseph disease (spinocerebellar ataxia type 3), a neurodegenerative disorder caused by triplet repeat expansion.
• UniProt keywords highlight additional roles in neurodegeneration, transcription regulation, and ubiquitin conjugation pathways.

Experimental Guidance and Technical Tips

• The recombinant immunogen spanning amino acids 1–361 of human ATXN3 includes the conserved N-terminal Josephin domain; consider sequence alignment when assessing cross-species reactivity (validated for human, mouse, rat).
• For Western blotting, expect a band near 41 kDa; expanded polyglutamine repeats in disease models may cause shifts. Validate with appropriate cell or tissue lysates.
• In IHC-P and IF/ICC, optimize antigen retrieval and antibody dilution to reduce background inherent to polyclonal preparations.
• The antibody’s reactivity with human, mouse, and rat supports cross-species studies; always include species-specific positive and negative controls.

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CamelBio offers diagnostic manufacturers and research laboratories a streamlined, one-stop sourcing solution for IVD raw materials, including validated antibodies such as this anti-Ataxin-3 polyclonal antibody for neurodegenerative disease and ubiquitin pathway research. Our portfolio covers optimized monoclonal and polyclonal antibodies, bulk ancillary reagents, and rare target sourcing, providing you with the comprehensive supply chain support required from early-stage development to clinical application.