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Item No. 9001600

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4-Methylumbelliferyl-α-L-iduronide 2-sulfate (4-MU-α-IdoA 2-sulfate) is a fluorogenic substrate of α-L-iduronidase that has been used in an assay to detect Hurler syndrome.1 It is also used as a substrate for iduronate-2-sulfatase in tests for Hunter disease.2,3 For these latter tests, the initial enzymatic product, 4-MU-α-IdoA (Item No. 19543) can be measured by mass spectrometry, or it can be hydrolyzed with α-L-iduronidase to liberate the fluorophore 4-MU, which has an emission maximum at 445-454 nm. The excitation maximum for 4-MU is pH-dependent: 330, 370, and 385 nm at pH 4.6, 7.4, and 10.4, respectively.4
WARNING This product is not for human or veterinary use.
1. Hurler’s syndrome, an -
2. Direct assay of iduronate-
3. A fluorimetric enzyme assay for the diagnosis of MPS II (Hunter disease). J. Inherit. Metab. Dis. 24(6), 675-680 (2001).
4. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule. J. Spectrosc. 147128 (2013).