The product of this gene is a lysosomal enzyme found in all cells.
It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate.
Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome).
Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.
Applications:Suitable for use in Western Blot and Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 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：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.09% sodium azide, 50% glycerol.
Specificity：Recognizes human GNS. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Recombinant protein corresponding to human GNS.
