Carbon-13 labeled Globotriaosylsphingosine (LysoGb3-13C6), biomarker internal standard for deficiency of α-Galactosidase A (GLA).

The absolute amount of LysoGb3-13C6 in the vial was determined using quantitative NMR to have the stated value on the vial. The vial may have additional components, but none are isobaric with LysoGb3-13C6, and these additional components have no effect on the ability to quantify the LysoGb3-13C6.

LysoGb3 (13C6-labeled)

SKU: BM36A

13C6-Globotriaosylsphingosine

Catalog Number: BM36A

Chemical Formula: C30 13C6 H67 N O17

Molecular Weight: 791.87

Approximate Amount per Assay: 0.8 ng
Hong, X., Sadilek, M. and Gelb, M.H., 2020. 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, pp.1-7. DOI: 10.1038/s41436-020-0790-9

Boutin, M., Lavoie, P., Abaoui, M. and Auray‐Blais, C., 2016. Tandem Mass Spectrometry Quantitation of Lyso‐Gb3 and Six Related Analogs in Plasma for Fabry Disease Patients. Current protocols in human genetics, 90(1), pp.17-23.

Johnson, B., Mascher, H., Mascher, D., Legnini, E., Hung, C.Y., Dajnoki, A., Chien, Y.H., Maródi, L., Hwu, W.L. and Bodamer, O.A., 2013. Analysis of lyso-globotriaosylsphingosine in dried blood spots. Annals of laboratory medicine, 33(4), pp.274-278.

LysoGb3 (13C6-labeled)

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