货号 | 9001600-1mg |
描述 | 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 |
别名 | 4-Methylumbelliferyl-α-L-Idopyranosiduronic Acid 2-sulfate;4-MU-α-IdoA 2-sulfate;MU-α-IdoA 2-sulfate; |
供应商 | Cayman |
应用文献 | |
1.Matalon, R., and Dorfman, A. Hurler’s syndrome, an -L-iduronidase deficiency. Biochem. Biophys. Res. Commun. 47(4), 959-964 (1972). 2.Lee, K.,Jun, S.-H.,Song, S.H., et al. Direct assay of iduronate-2-sulfatase for Hunter disease using UPLC-tandem mass spectrometry and fluorogenic substrate. Clin. Biochem. 48(18), 1350-1353 (2015). 3.Voznyi, Y.V.,Keulemans, J.L., and Van Diggelen, O.P. A fluorimetric enzyme assay for the diagnosis of MPS II (Hunter disease). J. Inherit. Metab. Dis. 24(6), 675-680 (2001). 4.Zhi, H.,Wang, J.,Wang, S., et al. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule. J. Spectrosc. 2013, 1-9 (2014). | |
运输条件 | Wet ice in continental US; may vary elsewhere |
存放说明 | -20 |
纯度 | ≥98% |
计算分子量 | 476.3 |
分子式 | C16H14O12S • 2Na |
稳定性 | ≥ 2 years |
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