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Search Results for " sulphamidase "

1

抑制剂 & 化合物

2

重组蛋白

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Cat. No.	Product Name	Target	Signaling Pathways
T37568	4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt)
	4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (4-MU-α-GlcNS) is a fluorogenic substrate of heparin sulphamidase. Heparin sulphamidase cleaves 4-MU-α-GlcNS to yield 4-MU-α-GlcNH2, which is then cleaved by α-glucosaminidase to release the fluorescent product 4-MU, which displays an emission maxima of 445-454 nm. The excitation maxima for 4-MU is pH-dependent: 330, 370, and 385 nm at pH 4.6, 7.4, and 10.4, respectively. 4-MU-α-GlcNS has been used to quantify heparin sulphamidase defi...

化合物

4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (sodium salt)

Cat.No: T37568

Species：

Expression System：

Tag：

Cat. No.	Product Name	Species	Expression System
TMPJ-01002	SGSH Protein, Human, Recombinant (His) SGSH,N-Sulphoglucosamine Sulphohydrolase,Sulfoglucosamine Su...	Human	HEK293 Cells
	N-Sulphoglucosamine Sulphohydrolase (SGSH) is an important member of the sulfatase family which is involved in the degradation of heparin sulfate. SGSH binds one calcium ion per subunit as a cofactor. SGSH catalyzes N-sulfo-D-glucosamine and H2O to D-glucosamine and sulfate. SGSH deficiency is result in mucopolysaccharidosis type 3A (MPS3A), a recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations.
TMPY-00368	ALR Protein, Human, Recombinant (His) HPO,ERV1,HERV1,ALR,HSS,HPO2,growth factor, augmenter of live...	Human	HEK293 Cells
	Alterations in GFER gene have been associated with progressive mitochondrial myopathy, congenital cataracts, hearing loss, developmental delay, lactic acidosis and respiratory chain deficiency in 3 siblings born to consanguineous Moroccan parents by homozygosity mapping and candidate gene approach. Using homozygosity mapping, we discovered that a mutation in the GFER gene causes an infantile mitochondrial disorder.

重组蛋白

SGSH Protein, Human, Recombinant (His)

Cat.No: TMPJ-01002

Species: Human

Expression System: HEK293 Cells

ALR Protein, Human, Recombinant (His)

Cat.No: TMPY-00368

Species: Human

Expression System: HEK293 Cells

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