4-Methylumbelliferyl-alpha-D-glucopyranoside, 99%

Fluorogenic substrate for alpha-D-glucosidase. It has been used in studies of Pompe's disease (Glycogen Storage Disease). Used for differentiation and enumeration of Enterobacter sakazakii based on alpha-glucosidase activity. 1. Broadhead D.M., Butterworth J., Pompe's disease: diagnosis in kidney and leucocytes using 4-Methylumbelliferyl-alpha-D-glucopyranoside. Clin. Genet. 1978, 13, 504-10. 2. Butterworth J., Broadhead D.M., Diagnosis of Pompe's disease in cultured skin fibroblasts and primary amniotic fluid cells using 4-Methylumbelliferyl-alpha-D-glucopyranoside as substrate. Clin. Chim. Acta 1977, 78, 335-42. 3. Holzapfel-Pschorn A., et al., Sensitive methods for the determination of microbial activities in water samples using fluorogenic substrates. Fres. Z. Anal. Chem. 1987, 327, 521-. 4. Leuschner R.G., Bew J., J. AOAC Int. 2004, 87, 604-13. 5. Oh S.W., Kang D.H., Fluorogenic selective and differential medium for isolation of Enterobacter sakazakii. Appl. Environ. Microbiol. 2004, 70, 5692-4. 6. Okumiya T., Keulemans J.L., Kroos M.A., Van der Beek N.M., Boer M.A, Takeuchi H., Van Diggelen O.P., Reuser A.J., A new diagnostic assay for glycogen storage disease type II in mixed leukocytes. Mol. Genet. Metab. 2006, 88, 22-8. 7. Umapathysivam K., Hopwood J.J., Meikle P.J., Determination of acid alpha-glucosidase activity in blood spots as a diagnostic test for Pompe disease. Clin. Chem. 2001, 47, 1378-83. 8. Zhang H., Kallwass H., Young S.P., Carr C., Dai J., Kishnani P.S., Millington D.S., Keutzer J., Chen Y.T., Bali D., Comparison of maltose and acarbose as inhibitors of maltase-glucoamylase activity in assaying acid alpha-glucosidase activity in dried blood spots for the diagnosis of infantile Pompe disease. Genet. Med. 2006, 8, 302-6.