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 and Immunohistochemistry.
Other applications not tested.
Recommended Dilutions:Immunohistochemistry (FFPE): 1:50-1:100Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Peptide Synthesis Grade
Purity：Purified by ammonium sulfate precipitation
Form：Supplied as a liquid in PBS, 0.09% sodium azide.
Specificity：Recognizes human Ataxin 3.
Isotype：IgG
Calc Applications Abbrev：E IHC
Calc Crossreactivity：Hu
Immunogen：KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 29-59 in the N-terminal region of human Ataxin 3.
