Anti-Ataxin-3 (ATXN3) Rabbit Monoclonal Antibody for WB and ELISA - P54252

Core Product Specifications and Parameters

Biological Background: Ataxin-3 Function and Localization

• Ataxin-3, also known as Machado-Joseph disease protein 1 (MJD1), is a deubiquitinating enzyme involved in multiple cellular processes including protein homeostasis, transcription, and cytoskeleton regulation. Related references: PMID:12297501, PMID:16118278, PMID:17696782
• It binds and trims long polyubiquitin chains but has weak activity toward chains of four or fewer ubiquitins. Related references: PMID:17696782
• Through interaction with STUB1/CHIP, Ataxin-3 promotes degradation of misfolded chaperone substrates by restricting ubiquitin chain length on monoubiquitinated STUB1/CHIP.
• It functions in transcription regulation by acting as a histone-binding protein and interacting with transcription regulators to repress gene expression. Related references: PMID:12297501
• Ataxin-3 negatively regulates mTORC1 signaling by deubiquitinating RHEB, leading to its inactivation by the TSC-TBC complex in response to amino acid deprivation. Related references: PMID:33157014
• It regulates autophagy through deubiquitination and stabilization of BECN1. Related references: PMID:28445460
• Subcellularly, Ataxin-3 is found in the nucleus matrix, nucleus, and lysosome membrane, consistent with its roles in nuclear processes and lysosome-related functions.
• The ATXN3 gene is linked to spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, a neurodegenerative disorder characterized by triplet repeat expansion.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 190–361 of human Ataxin-3; this region is likely conserved, consistent with reported mouse and rat cross-reactivity. Consider verifying reactivity in your sample system.
• For WB, test a range of dilutions using whole-cell lysates from human, mouse, or rat neuronal cell lines or Machado-Joseph disease models.
• In ELISA applications, pair this antibody with a detection antibody that recognizes a non-overlapping epitope, or use it as a capture/detection component in sandwich assays.
• As a recombinant monoclonal antibody, it offers consistent lot-to-lot performance, suitable for quantitative and reproducible experiments.

CamelBio: Your One-Stop Sourcing Bridge

At CamelBio, we specialize in supplying high-quality IVD raw materials and technical support to accelerate your diagnostic and research programs. This anti-Ataxin-3 monoclonal antibody exemplifies our commitment to providing validated reagents for neurodegeneration and protein homeostasis studies. Contact us to explore our complete portfolio of antibody pairs, bulk ancillary reagents, and customized sourcing solutions.