4-Methylumbelliferyl 2-Sulfamino-2-deoxy-α-D-glucopyranoside Sodium Salt

4-MU-α-GlcNS sodium is a fluorogenic substrate of heparin sulphamidase, is desulfurized into 4-MU-α-GlcNH2. 4-MU-α-GlcNH2 can liberate 4-methylumbelliferone (4-MU, fluorescent product) via α-glucosaminidase catalysis, with the emission wavelength maxima of 445-454 nm. 4-MU-α-GlcNS sodium can be used to heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders researches[1][2][3].

Research Areas >> Others

Signaling Pathways >> Others >> Others

[1]. Karpova EA, et al. A fluorimetric enzyme assay for the diagnosis of Sanfilippo disease type A (MPS IIIA). J Inherit Metab Dis. 1996;19(3):278-85. 

[2]. Dasgupta F, et al. Synthesis of 7-O-(2-deoxy-2-sulfamido-alpha-D-glucopyranosyl)-4-methylcoumarin sodium salt: a fluorogenic substrate for sulfamidase. Carbohydr Res. 2002 Jun 5;337(11):1055-8. 

[3]. G. Civallero, et al. Assay of heparan-N-sulfamidase in dried leukocytes impregnated in filter paper: A new tool for the identification of Mucopolisaccharidosis IIIA and potentially other lysosomal disorders, Molecular Genetics and Metabolism. 2013;108(4): 267-268.