4-Methylumbelliferyl alpha-D-glucopyranoside
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Beschreibung4-Methylumbelliferyl-α-D-glucopyranoside is a fluorogenic substrate for α-glucosidase. 4-Methylumbelliferyl-α-D-glucopyranoside is cleaved by α-glucosidase 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 pH (7.12-10.3), respectively, and an emission maximum ranging from 445 to 455 nm, increasing as pH decreases. 4-Methylumbelliferyl-α-D-glucopyranoside has been used to quantify α-glucosidase activity in infant blood spot samples as a biomarker of Fabry and Pompe diseases, lysosomal storage disorders characterized by a deficiency in the enzyme.
Verwenden4-Methylumbelliferyl-α-D-glucopyranoside is a fluorogenic substrate in the assay of a-glucosidase,it has been used in the study of Pompe disease.
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