4-<wbr/>Methyl<wbr/>umbelliferyl-?-<wbr/>L-<wbr/>Iduronide (free acid)

4-Methylumbelliferyl-?-L-Iduronide (free acid)

CAT N°: 19543
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From 92.00 78.20

4-Methylumbelliferyl-?-L-iduronide (free acid) is a fluorogenic substrate for ?-L-iduronidase, an enzyme found in cell lysosomes that is involved in the degradation of glycosaminoglycans such as dermatan sulfate and heparin sulfate. 4-Methylumbelliferyl-?-L-iduronide is cleaved by ?-L-iduronidase to release the fluorescent moiety 4-methylumbelliferyl (4-MU). 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high (7.12-10.3) pH, respectively, and an emission maximum ranging from 445 to 455 nM, increasing as pH decreases.{27251} This substrate is used in assays that measure the activity of ?-L-iduronidase, which is commonly deficient in a type of lysosomal storage disease called mucopolysaccharidosis.{31700}

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