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 ELISA and Western Blot.
Other applications not tested.
Recommended Dilution:ELISA: 1:1,000Western Blot: 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 at least 12 months.
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：Purified
Purity：Purified by ammonium sulfate precipitation.
Form：Supplied as a liquid in PBS, 0.09% sodium azide.
Specificity：Recognizes human GNS.
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu
Immunogen：Synthetic peptide selected from the Center region of human GNS (KLH).
