|Application ||WB, IHC-P, E|
|Other Accession||Q8BFR4, Q1LZH9|
|Calculated MW||62082 Da|
|Antigen Region||174-203 aa|
|Other Names||N-acetylglucosamine-6-sulfatase, Glucosamine-6-sulfatase, G6S, GNS|
|Target/Specificity||This GNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the Central region of human GNS.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||GNS Antibody (Center N189) is for research use only and not for use in diagnostic or therapeutic procedures.|
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Provided below are standard protocols that you may find useful for product applications.
GNS 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.
Zhang,H., Li,X.J., Nat. Biotechnol. 21 (6), 660-666 (2003)
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