Details and applications of our offerings (both sets and individuals) for MS/MS screening and tuning are noted in the resources and referencs below. In particular, the
MS/MS Screening Standards catalog lists the composition details and reconstitution recommendations (for concentrated and working stocks), but also the usage specifications (i.e., storage and recommended retest date before and after reconstitution) for our reference standard sets. The sets contain a collection of stable isotope-labeled standards (e.g., 12 amino acids in NSK-A) and are available in a 10-vial set or as single vials. CIL also has the ability to customize standards or mixes to meet your specific needs. Please inquire for details.
Frequently Asked Questions
What is the recommended procedure for solubilizing NSK-A and -B?
The lyophilized amino acid mix in NSK-A (and NSK-A1) should be dissolved in 1 mL of 50:50 purified water:methanol and the carnitine/acylcarnitines in NSK-B (and NSK-B-G1) in 1 mL of purified methanol. Following the solvent addition, each vial should be vortexed manually for 1 min then auto-vortexed for 30 min (or longer until fully solubilized).
Are the component chemical purities considered in the NSK product formulations?
Yes, the chemical purity of each component (as stated on the CoA) is reviewed at time of formulation and the gravimetry is adjusted as necessary to compensate for chemical purity.
What concentration should I use on the CoA to represent the standard in use – gravimetric target concentration, concentration by gravimetry, or analyzed concentration?
The concentrations presented in the “Concentration by Gravimetry” column of the CoA should be used to represent the actual concentration of each component after reconstitution.
References
Schupper, A.; Almashanu, S.; Coster, D.; et al.
2021. Metabolic biomarkers of small and large for gestational age newborns.
Early Hum Dev, 160, 105422.
PMID: 34271419
Young, A.; Hendricks, J.; Foreman, D.; et al.
2020. Development of dried blood spot quality control materials for adenosine deaminase severe combined immunodeficiency and an LC-MS/MS method for their characterization.
Clin Mass Spec, 17, 4-11.
PMID 33851028
Staretz-Chacham, O.; Daas, S.; Ulanovsky, I.; et al.
2021. The role of orotic acid measurement in routine newborn screening for urea cycle disorders.
J Inherit Metab Dis, 44(3), 606-617.
PMID: 33190319
Lai, F.; Srinivasan, S.; Wiley, V.
2020. Evaluation of a two-tier screening pathway for congenital adrenal hyperplasia in the New South Wales Newborn Screening Programme.
Int J Neonatal Screen, 6(3), 63.
PMID: 33117905
Yang, Y.; Wu, Z.; Li, S.; et al.
2020. Targeted blood metabolomic study on retinopathy of prematurity.
Invest Ophthalmol Vis Sci, 61(2), 12.
PMID: 32049343
Brennenstuhl, H.; Kohlmüller, D.; Gramer, G.; et al.
2020. High throughput newborn screening for aromatic ʟ-amino-acid decarboxylase deficiency by analysis of concentrations of 3-O-methyldopa from dried blood spots.
J Inherit Metab Dis, 43(3), 602-610.
PMID: 31849064
Sun, R.; Li ,Y.; Cai, M.; et al.
2019. Discovery of a new biomarker pattern for differential diagnosis of acute ischemic stroke using targeted metabolomics. Front
Neurol, 10, 1011.
PMID: 31608005
Matthew, E.M.; Lewis, L.; Rao, P.; et al.
2019. Novel HILIC-ESI-MS method for urinary profiling of MSUD and methylmalonic aciduria biomarkers.
J Chromatogr Sci, 57(8), 715-723.
PMID: 31251316
Jack, R.M.; Scott, C.R.
2019. Validation of a therapeutic range for nitisinone in patients treated for tyrosinemia type 1 based on reduction of succinylacetone excretion.
JIMD Rep, 46(1), 75-78.
PMID: 31240158
Bai, Q.; Peng, B.; Wu, X.; et al.
2018. Metabolomic study for essential hypertension patients based on dried blood spot mass spectrometry approach.
IUBMB Life, 70(8), 777-785.
PMID: 30092118
Jacob, M.; Malkawi, A.; Albast, N.; et al.
2018. A targeted metabolomics approach for clinical diagnosis of inborn errors of metabolism.
Anal Chim Acta, 1025, 141-153.
PMID: 29801603
Coene, K.L.M.; Kluijtmans, L.A.J.; van der Heeft, E.; et al.
2018. Next-generation metabolic screening: targeted and untargeted metabolomics for the diagnosis of inborn errors of metabolism in individual patients.
J Inherit Metab Dis,
41(3), 337-353.
PMID: 29453510
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.
doi.org/10.1177%2F2326409817692360.
Céspedes, N., Valencia, A., Echeverry, C.A., et al.
2017. Reference values of amino acids, acylcarnitines and succinylacetone by tandem mass spectrometry for use in newborn screening in southwest Colombia.
Colomb Med,
48(3), 113-119.
PMID: 29213153
de Sain-van der Velden, M.G.M.; van der Ham, M.; Gerrits, J.; et al.
2017. Quantification of metabolites in dried blood spots by direct infusion high resolution mass spectrometry.
Anal Chim Acta,
979, 45-50.
PMID: 28599708
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 ass spectrometry.
Clin Chem, 62(9), 1248-1254.
PMID: 27440509
Huang, T.; Cao, Y.; Zeng, J.; et al.
2016. Tandem mass spectrometry-based newborn screening strategy could be used to facilitate rapid and sensitive lung cancer diagnosis.
Onco Targets Ther,
9, 2479-2487.
PMID: 27217771
Prinsen, H.C.M.T.; Schiebergen-Bronkhorst, B.G.M.; Roeleveld, M.W.; et al.
2016. Rapid quantification of underivatized amino acids in plasma by hydrophilic interaction liquid chromatography (HILIC) coupled with tandem mass spectrometry.
J Inherit Metab Dis, 39(5), 651-660.
PMID: 27099181
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.
PMID: 26707915
George, R.S.; Moat, S.J.
2016. Effect of dried blood spot quality on newborn screening analyte concentrations and recommendations for minimum acceptance criteria for sample analysis.
Clin Chem,
62(3), 466-475.
PMID: 26647314
Haynes, C.A.; De Jesús, V.R.
2016. Simultaneous quantitation of hexacosanoyl lysophosphatidylcholine, amino acids, acylcarnitines, and succinylacetone during FIA-ESI-MS/MS analysis of dried blood spot extracts for newborn screening.
Clin Biochem,
49(1-2), 161-165.
PMID: 26432925
Miller, M.J.; Kennedy, A.D.; Eckhart, A.D.; et al.
2015. Untargeted metabolomic analysis for the clinical screening of inborn errors of metabolism.
J Inherit Metab Dis,
38(6), 1029-1039.
PMID: 25875217
Held, P.K.; Haynes, C.A.; De Jesús, V.R.; et al.
2014. Development of an assay to simultaneously measure orotic acid, amino acids, and acylcarnitines in dried blood spots.
Clin Chem Acta,
436, 149-154.
PMID: 24886687
Chace, D.H.; Kalas, T.A.; Naylor, E.W.
2003. Use of tandem mass spectrometry for multianalyte screening of dried blood specimens from newborns.
Clin Chem,
49, 1797-1817.
PMID: 14578311
MS/MS Screening Mixtures and Standards
