Substrate and Internal Standard Mixes

*Code S-IS24  Valid for domestic direct and international direct customers only. Offer expires 12/31/24, while supplies last. Other exclusions may apply.

CIL is pleased to offer six dried-down mixtures of substrate (S) and internal standard (IS) combinations for use in basic and/or translational MS/MS screening research.

NSK-FA α-Galactosidase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 500:1.

Substrate
(6-Benzoylamino-hexyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]-ethyl}-carbamic acid tert-butyl ester
Chemical Formula: C33H47N3O10   MW: 645.7 g/mol

Internal Standard
(6-D5-Benzoylamino-hexyl)-[2-(4-hydroxy-phenyl-carbamoyl)-ethyl]-carbamic acid tert-butyl ester
Chemical Formula: C27H32N3O5D5   MW: 488.5 g/mol

NSK-GA Glucocerebrosidase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 50:1.

Substrate
D-Glucosyl-β1-1‘-N-dodecanoyl-D-erythro-sphingosine [C12-glucocerebroside]
Chemical Formula: C36H69NO8   MW: 643.9 g/mol

Internal Standard
N-Myristoyl-D-erythro-sphingosine [C14-ceramide]
Chemical Formula: C32H63NO3   MW: 509.8 g/mol

NSK-KR Galactocerebrosidase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 150:1.

Substrate
D-Galactosyl-β1-1’-octanoyl-D-erythro-sphingosine [C8-galactosylceramide]
Chemical Formula: C32H61NO8   MW: 587.8 g/mol

Internal Standard
N-Decanoyl-D-erythro-sphingosine [C10-ceramide]
Chemical Formula: C28H55NO3   MW: 453.7 g/mol

NSK-MP α-L-Iduronidase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 150:1.

Substrate
(7-(1-Iduronic acid)-oxycoumarin-4-methylamine-(5’-N-boc-aminopentanoyl)-amide)
Chemical Formula: C26H34N2O12   MW: 566.6 g/mol

Internal Standard
(7-Hydroxycoumarin-4-methylamine-(4’-N-boc-aminobutanoyl)-amide)
Chemical Formula: C19H24N2O6   MW: 376.4 g/mol

NSK-NI Acid Sphingomyelinase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 50:1.

Substrate
N-Hexanoyl-D-erythro-sphingosylphosphorylcholine [C6-sphingomyelin]
Chemical Formula: C29H59N2O6P   MW: 562.8 g/mol

Internal Standard
N-Butyroyl-D-erythro-sphingosine [C4-ceramide]
Chemical Formula: C22H43NO3   MW: 369.6 g/mol

NSK-PO Acid α-Glucosidase Substrate and Internal Standard Mix

Each vial contains the following compounds at a molar ratio of 100:1.

Substrate
(7-Benzoylamino-heptyl)-{2-[4-(3,4,5-trihydroxy-6-hydroxymethyl-tetrahydro-pyran-2-yloxy)-phenylcarbamoyl]-ethyl}-carbamic acid tert-butyl ester
Chemical Formula: C34H49N3O10   MW: 659.8 g/mol

Internal Standard
(7-D5-Benzoylamino-heptyl)-[2-(4-hydroxy-phenyl-carbamoyl)-ethyl]-carbamic acid tert-butyl ester
Chemical Formula: C28H34N3O5D5   MW: 502.7 g/mol

Frequently Asked Questions

What is the shelf life of these S/IS mixes? In their supplied dried-down form, the stored S/IS mixes at -20° (light protected) are stable for 2 years from their date of manufacture. Upon reconstitution, the S and IS compounds are stable at 5±3ºC or -20±5ºC for 4 weeks.

What is an appropriate solvent for solubilizing these S/IS mixes? While we do not provide instructions for preparing these mixes for sample application, dissolution solvents and assay preparations are described in the research literature (see published references below for examples).

Can alternate S/IS mixtures be custom formulated? We offer the ability to customize. However, we would first need to review feasibility. To start this process, please provide the necessary details on this Custom Mix Request form or contact your local sales representative for a quote.

Example References

Ribas, G.; De Mari, J.F.; Civallero, G.; et al. 2017. Validation of a multiplex tandem mass spectrometry method for the detection of selected lysosomal storage diseases in dried blood spots. JIMES, 5, 1-7.
Tortorelli, S.; Turgeon, C.T.; Gavrilov, D.K.; et al. 2016. Simultaneous testing for 6 lysosomal storage disorders and x-adrenoleukodystrophy in dried blood spots by tandem mass spectrometry. Clin Chem, 62(9), 1248-1254.
Cho, S.E.; Kwak, J.R.; Lee, H.; et al. 2016. Triplex tandem mass spectrometry assays for the screening of 3 lysosomal storage disorders in a Korean population. Clin Chim Acta, 454, 20-27.