|Application ||IHC-P, E|
|Calculated MW||41781 Da|
|Antigen Region||29-59 aa|
|Other Names||Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3|
|Target/Specificity||This Ataxin3 (MJD) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 29-59 amino acids from the N-terminal region of human Ataxin3 (MJD).|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||Ataxin3 (MJD) Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Synonyms||ATX3, MJD, MJD1, SCA3|
|Function||Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone- binding protein that regulates transcription.|
|Cellular Location||Nucleus matrix. Note=Predominantly nuclear, but not exclusively, inner nuclear matrix|
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Provided below are standard protocols that you may find useful for product applications.
Machado-Joseph disease is an autosomal dominant neurologic disorder, and is now known to be the same as previously described spinocerebellar ataxia-3. MJD protein (Ataxin-3) contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. This protein interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription, and also acts as a histone-binding protein that regulates transcription. MJD is a deubiquitinating enzyme.
Albrecht, M., et al., Eur. J. Biochem. 271(15):3155-3170 (2004).
Michlewski, G., et al., J. Mol. Biol. 340(4):665-679 (2004).
Li, Y., et al., Ann. Neurol. 56(1):124-129 (2004).
Beuckmann, C.T., et al., J. Neurosci. 24(18):4469-4477 (2004).
Berke, S.J., et al., J. Neurochem. 89(4):908-918 (2004).
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