Substrate for mass spectrometric assay of the lysosomal enzyme α-Galactosidase A (GLA).

α-Galactosidase A Substrate for MS/MS Assays

SKU: SM33A

t-butyl (6-benzamidohexyl){3-[(4-hydroxyphenyl)amino]-3-oxopropyl}carbamate-α-galactoside

Catalog Number: SM33A

Chemical Formula: C33 H47 N3 O10

Molecular Weight: 645.74

Approximate Amount per Assay: 24 µg
Liao, H.C., Chan, M.J., Yang, C.F., Chiang, C.C., Niu, D.M., Huang, C.K. and Gelb, M.H., 2017. Mass spectrometry but not fluorimetry distinguishes affected and pseudodeficiency patients in newborn screening for Pompe disease. Clinical Chemistry, 63(7), pp.1271-1277.

Zhang, X.K., Elbin, C.S., Turecek, F., Scott, R., Chuang, W.L., Keutzer, J.M. and Gelb, M., 2010. Multiplex lysosomal enzyme activity assay on dried blood spots using tandem mass spectrometry. Methods Mol Biol. 603, pp.(339-50).

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