4-Methylumbelliferyl-β-D-glucopyranoside (4-MU-β-D-Glc) is a sensitive fluorogenic substrate for β-glucosidase and β-glucocerebrosidase (also known as glucosylceramidase) yielding a blue fluorescent solution. Employed in the detection of beta-glucosidase as an indicator of Enterococci. 4-MU-β-D-Glc has been extensively used in work on Gaucher's disease, a lipid storage disease characterized by the accumulation of glucocerebroside due to a genetic deficiency of a β-glucosidase, glucosylceramidase. A test for Gaucher's disease, has been reported whereby 4-MU-β-D-Glc is hydrolyzed by glucosylceramidase in the presence of sodium taurocholate, which suppresses the activity of other β-glucosidases. Hydrolysis of 4-MU-β-D-Glc releases the fluorescent product 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-Methylumbelliferyl-β- D-glucopyranoside and other fluorogenic substrates have been used to compare normal acid hydrolase activity with that of patients with various forms of mucopolysaccharidoses and in a study of four acid hydrolases in human kidney and urine. The use of 4-MU-β-D-Glc has also been reported in a rapid method for identifying bacterial enzymes.
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