Substrate for mass spectrometric assay of the lysosomal enzyme Acid α-glucosidase (GAA).

Acid α-glucosidase Substrate for MS/MS Assays

SKU: SM34B

t-butyl (7-benzamidoheptyl){3-[(4-hydroxyphenyl)amino]-3-oxopropyl}carbamate-α-glucoside

Catalog Number: SM34B

Chemical Formula: C34 H49 N3 O10

Molecular Weight: 659.77

Approximate Amount per Assay: 7 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.

Hong, Xinying, Martin Sadilek, and Michael H. Gelb. "A highly multiplexed biochemical assay for analytes in dried blood spots: application to newborn screening and diagnosis of lysosomal storage disorders and other inborn errors of metabolism." Genetics in Medicine 22, no. 7 (2020): 1262-1268.

Elliott, Susan, Norman Buroker, Jason J. Cournoyer, Anna M. Potier, Joseph D. Trometer, Carole Elbin, Mack J. Schermer et al. "Pilot study of newborn screening for six lysosomal storage diseases using Tandem Mass Spectrometry." Molecular genetics and metabolism 118, no. 4 (2016): 304-309.

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