N-acetylgalactosamine-6-sulfatase is an enzyme that, in humans, is encoded by the GALNS gene
This gene encodes N-acetylgalactosamine-6-sulfatase, which is a lysosomal exohydrolase required for the degradation of the glycosaminoglycans, keratan sulfate, and chondroitin 6-sulfate.
Sequence alterations including point, missense and nonsense mutations, as well as those that affect splicing, result in a deficiency of this enzyme.
Deficiencies of this enzyme lead to Morquio A syndrome, a lysosomal storage disorder.
Applications:Suitable for use in ELISA and Western Blot.
A band is detcted at ~60kD in Human Bone Marrow and Placenta lysates.
Optimal dilutions to be determined by the researcher.
Peptide Blocking:Corresponding peptide is available for Peptide Blocking studies.
See G1024-01-P.
Antibody is typically 0.5mg/ml and peptide is supplied as a 100ul pellet.
When peptide is reconstituted in 200ul water, the concentration would also be 0.5mg/ml.
To start, the best ratio would be 1:1 (molar excess of peptide relative to antibody when identical volumes are mixed).
Mix equal volumes of peptide and antibody at the required dilution and leave at ambient temperature.
It is best is to have two identical blots to be incubated with equal amount of antibody, but one with the antibody pre-adsorbed to the peptide for 20min.
Then incubate and develop the two blots in parallel.
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 at least 12 months.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ug
Host：goat
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by peptide affinity chromatography.
Form：Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.
Specificity：Recognizes human GALNS.
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide corresponding to the C-terminus, TTHNLEDHTKLP of human GALNS.
