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An effective substrate for the detection of N-acetyl-a-D-Galactopyranosiaminidase activity. The deficiency of this enzyme is the defect in Schindler disease

An effective substrate for the detection of N-acetyl-α-D-Galactopyranosiaminidase activity. The deficiency of this enzyme is the defect in Schindler disease.

4-Methylumbelliferyl 2-acetoamido-2-deoxy-α-D-galactopyranoside (4-MU-2-acetoamido-2-deoxy-α-Gal) is a fluorogenic substrate used to quantify α- and β-galactopyranosaminidase activity. 4-MU-2-acetoamido-2-deoxy-α-Gal is cleaved by α- and β-galactopyranosaminidase to release the fluorescent moiety 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.

[1] R. SZWEDA. Synthesis of 4-methylumbelliferyl glycosides for the detection of α- and β-D-galactopyranosaminidases[J]. Canadian Journal of Chemistry, 1989, 22 1: 1388-1391. DOI: 10.1139/v89-213
[2] H. ZHI. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule[J]. Spectroscopy, 2013, 438 1: 1-9. DOI: 10.1155/2013/147128