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Item No. 24931

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4-Methylumbelliferyl 2-sulfamino-2-deoxy-α-D-Glucopyranoside (4-MU-α-GlcNS) is a fluorogenic substrate of heparin sulphamidase.1 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.1,2 The excitation maxima for 4-MU is pH-dependent: 330, 370, and 385 nm at pH 4.6, 7.4, and 10.4, respectively.2 4-MU-α-GlcNS has been used to quantify heparin sulphamidase deficiencies associated with Mucopolisaccaridosis IIIA and other lysosomal disorders.3
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1. A fluorimetric enzyme assay for the diagnosis of Sanfilippo disease type A (MPS IIIA). J. Inherit. Metab. Dis. 19(3), 278-285 (1996).
2. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule. J. Spectrosc. 147128 (2013).
3. Assay of heparan-