Internal standard (qNMR-quantified) for mass spectrometric assay of the lysosomal enzyme Acid α-glucosidase (GAA).

Acid α-glucosidase Internal Standard for MS/MS Assays

SKU: HA28A

t-butyl (7-benzamidoheptyl){3-[(4-hydroxyphenyl)amino]-3-oxopropyl}carbamate-d5

Catalog Number: HA28A

Chemical Formula: C28 H34 D5 N3 O5

Molecular Weight: 502.66

Approximate Amount per Assay: 360 ng
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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