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 FLISA and Immunohistochemistry.
Other applications not tested.
Recommended Dilutions:Immunohistochemistry: Formalin fixed and paraffin embedded tissuesOptimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Caution: FITC conjugates are sensitive to light.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A/G affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).
Specificity：Recognizes human Ataxin 3.
Isotype：IgG
Calc Applications Abbrev：FLISA 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.
