SGSH

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£320.00

SKU:: 334101
Reinheit:: Purified by immunoaffinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant protein corresponding to aa273-502 from human SGSH.
Format:: Supplied as a liquid in PBS, pH 7.3, 0.02% sodium azide, 50% glycerol.
Weitere Details:: SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62kD precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A. Applications: Suitable for use in ELISA and Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunohistochemistry (Paraffin): 1:20-1:200 Optimal 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.