Technical Data
A4159-09
Ataxin 3 (Ataxin-3, Machado-Joseph Disease Protein 1, Spinocerebellar Ataxia Type 3 Protein, ATXN3, ATX3, MJD, MJD1, SCA3)
Description:
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.

Applications:
Suitable for use in ELISA. Other applications not tested.

Recommended Dilution:
ELISA: 1:100-1:1000
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
TypeIsotypeCloneGrade
MabIgG1,k11E21Affinity Purified
SizeStorageShippingSourceHost
100ug-20CBlue IceHumanMouse
Concentration:
~0.5mg/ml
Immunogen:
Full length recombinant corresponding to ATX-3.
Purity:
Purified by Protein G affinity chromatography.
Form
Supplied as a liquid in PBS, 0.2% gelatin, 0.05% sodium azide.
Specificity:
Recognizes human ATX-3.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Warrick,J.M., Morabito,L.M., Bilen,J., Gordesky-Gold,B., Faust,L.Z., Paulson,H.L. and Bonini,N.M. Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Mol. Cell 18 (1), 37-48 (2005). 2. Burnett,B.G. and Pittman,R.N. The polyglutamine neurodegenerative protein ataxin 3 regulates aggresome formation. Proc. Natl. Acad. Sci. U.S.A. 102 (12), 4330-4335 (2005).