4-Methylumbelliferyl-alpha-L-Iduronide2-sulfate(sodiumsalt)

4-Methylumbelliferyl a-L-idopyranosiduronic acid 2-sulphate disodium salt; 4-MU-a-IdoA 2-sulfate;  4MU-a-L-iduronide-2-sulphate Na; M2; 4MU-alpha-L-iduronide-2-sulphate is a fluorogenic substrate for iduronate-2-sulphatase (IDS), used in a 2-step assay coupled with alpha-L-iduronidase. The assay is used in biochemical testing for Hunter syndrome (mucopolysaccharidosis II / MPS II). In a first step, iduronidate-2-sulphatase from biological samples cleaves the sulphate group from 4MU-alpha-L-iduronide-2-sulphate. In a second step, exogeneous alpha-L-iduronidase which cleaves the 4-MU moiety is added to the reaction, resulting in release of a fluorescent signal (Ex 360 nm / Em 455 nm). The reaction is stopped by the addition of sodium carbonate. 4-Methylumbelliferyl-?-L-iduronide 2-sulfate (4-MU-?-IdoA 2-sulfate) is a fluorogenic substrate of ?-L-iduronidase that has been used in an assay to detect Hurler syndrome. It is also used as a substrate for iduronate-2-sulfatase in tests for Hunter disease. For these latter tests, the initial enzymatic product, 4-MU-?-IdoA can be measured by mass spectrometry, or it can be hydrolyzed with ?-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. CAS Number 1045020-74-3 Product Name 4-Methylumbelliferyl-alpha-L-Iduronide 2-sulfate (sodium salt) IUPAC Name sodium;[6-carboxy-4,5-dihydroxy-2-(4-methyl-2-oxochromen-7-yl)oxyoxan-3-yl] sulfate Molecular Formula C16H15NaO12S Molecular Weight 476.3 InChI InChI=1S/C16H16O12S.Na/c1-6-4-10(17)26-9-5-7(2-3-8(6)9)25-16-14(28-29(22,23)24)12(19)11(18)13(27-16)15(20)21;/h2-5,11-14,16,18-19H,1H3,(H,20,21)(H,22,23,24);/q;+1/p-1 InChI Key BGWMBPGUPUSUIU-CEWNINSASA-L SMILES CC1=CC(=O)OC2=C1C=CC(=C2)OC3C(C(C(C(O3)C(=O)O)O)O)OS(=O)(=O)[O-].[Na+] Synonyms 4-Methylumbelliferyl-?-L-Idopyranosiduronic Acid 2-sulfate;4-MU-?-IdoA 2-sulfate;MU-?-IdoA 2-sulfate