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: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.
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：Purified
Purity：Purified by ammonium sulfate precipitation.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
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).
