Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder.
The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease.
There is a negative correlation between the age of onset and CAG repeat numbers.
Alternatively spliced transcript variants encoding different isoforms have been described for this gene.
Applications:Suitable for use in Immunofluorescence and Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunofluorescence: 1:50-1:200Optimal 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:100ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by affinity chromatography.
Form:Supplied as a liquid in PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
Specificity:Recognizes human ATXN3 at ~50kD. Species Crossreactivity: mouse, rat
Isotype:IgG
Calc Applications Abbrev:IF WB
Calc Crossreactivity:Hu Mo Rt
Immunogen:Recombinant protein corresponding to human ATXN3.