GNS / G6S 抗体

GNS / G6S 抗体产品信息

免疫原 :
Recombinant human GNS protein ( Catalog#10319-H08H )
抗体宿主 : Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
Produced in rabbits immunized with purified, human cell-derived, recombinant human GNS / G6S (rhGNS; Catalog#10319-H08H; NP_002067.1; Met 1- Leu 552). Total IgG was purified by Protein A affinity chromatography.
GNS / G6S 抗体Background
Human glucosamine (N-acetyl)-6-sulfatase (GNS), also known as G6S, is a lysosomal enzyme ubiquitously present in cells. GNS is required for the catabolism of the glycosaminoglycans (GAG) including heparin, heparan sulphate, and keratan sulphate through the hydrolysis of 6-sulfate group from the N-acetyl-D-glucosamine 6-sulfate units. The deficiency or loss-of-function of GNS result in mucopolysaccharidosis type IIID (MPS IIID), an inborn catabolism disorder with the accumulation of undegraded substrate and the lysosomal storage. As the least common type of the four subtypes of Sanfilippo syndrome, MPS IIID has profound mental deterioration, hyperactivity, and relatively mild somatic manifestations.
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