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A new simple and rapid LC–ESI-MS/MS method for quantification of plasma oxysterols as dimethylaminobutyrate esters. Its successful use for the diagnosis of Niemann–Pick type C disease
- Source :
- Clinica Chimica Acta. 437:93-100
- Publication Year :
- 2014
- Publisher :
- Elsevier BV, 2014.
-
Abstract
- Two oxysterols, cholestan-3β,5α,6β-triol (C-triol) and 7-ketocholesterol (7-KC), have been recently proposed as diagnostic markers of Niemann-Pick type C (NP-C) disease, representing a potential alternative diagnostic tool to the more invasive and time consuming filipin test in cultured fibroblasts. Usually, the oxysterols are detected and quantified by liquid chromatography-tandem mass spectrometry (LC-MS/MS) method using atmospheric pressure chemical ionization (APCI) or electro-spray-ionization (ESI) sources, after a variety of derivatization procedures to enhance sensitivity. We developed a sensitive LC-MS/MS method to quantify the oxysterols in plasma as dimethylaminobutyrate ester, suitable for ESI analysis. This method, with an easy liquid-phase extraction and a short derivatization procedure, has been validated to demonstrate specificity, linearity, recovery, lowest limit of quantification, accuracy and precision. The assay was linear over a concentration range of 0.5-200ng/mL for C-triol and 1.0-200ng/mL for 7-KC. Intra-day and inter-day coefficients of variation (CV%) were
- Subjects :
- Adult
Male
Spectrometry, Mass, Electrospray Ionization
Accuracy and precision
Time Factors
Lc esi ms ms
Adolescent
Clinical Biochemistry
Atmospheric-pressure chemical ionization
Mass spectrometry
Biochemistry
Young Adult
chemistry.chemical_compound
Liquid chromatography–mass spectrometry
Humans
Child
Derivatization
Ketocholesterols
Detection limit
Chromatography
Chemistry
Aminobutyrates
Biochemistry (medical)
Infant, Newborn
Infant
Niemann-Pick Disease, Type C
General Medicine
Child, Preschool
Niemann-pick type c disease
Female
lipids (amino acids, peptides, and proteins)
Biomarkers
Cholestanols
Chromatography, Liquid
Subjects
Details
- ISSN :
- 00098981
- Volume :
- 437
- Database :
- OpenAIRE
- Journal :
- Clinica Chimica Acta
- Accession number :
- edsair.doi.dedup.....27bfe322bd1d2914a2c484eb091ee58f