Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.18/5462
Título: Simultaneous quantitation of sphingoid bases by UPLC-ESI-MS/MS with identical13C-encoded internal standards
Autor: Mirzaian, M.
Wisse, P.
Ferraz, M.J.
Marques, A.R.A.
Gaspar, P.
Oussoren, S.V.
Kytidou, K.
Codée, J.D.C.
van der Marel, G.
Overkleeft, H.S.
Aerts, J.M.
Palavras-chave: Carbon Isotopes
Chromatography, High Pressure Liquid
Fabry Disease
Female
Gaucher Disease
Humans
Male
Reference Standards
Sphingolipidoses
Sphingolipids
Tandem Mass Spectrometry
Doenças Genéticas
Data: Mar-2017
Editora: Elsevier
Citação: Clin Chim Acta. 2017 Mar;466:178-184. doi: 10.1016/j.cca.2017.01.014. Epub 2017 Jan 13.
Resumo: Free sphingoid bases (lysosphingolipids) of primary storage sphingolipids are increased in tissues and plasma of several sphingolipidoses. As shown earlier by us, sphingoid bases can be accurately quantified using UPLC-ESI-MS/MS, particularly in combination with identical13C-encoded internal standards. The feasibility of simultaneous quantitation of sphingoid bases in plasma specimens spiked with a mixture of such standards is here described. The sensitivity and linearity of detection is excellent for all examined sphingoid bases (sphingosine, sphinganine, hexosyl-sphingosine (glucosylsphingosine), hexosyl2-sphingosine (lactosylsphingosine), hexosyl3-sphingosine (globotriaosylsphingosine), phosphorylcholine-sphingosine) in the relevant concentration range and the measurements show very acceptable intra- and inter-assay variation (<10% average). Plasma samples of a series of male and female Gaucher Disease and Fabry Disease patients were analyzed with the multiplex assay. The obtained data compare well to those earlier determined for plasma globotriaosylsphingosine and glucosylsphingosine in GD and FD patients. The same approach can be also applied to measure sphingolipids in the same sample. Following extraction of sphingolipids from the same sample these can be converted to sphingoid bases by microwave exposure and subsequently quantified using13C-encoded internal standards.
Peer review: yes
URI: http://hdl.handle.net/10400.18/5462
DOI: 10.1016/j.cca.2017.01.014
ISSN: 0009-8981
Versão do Editor: https://www.sciencedirect.com/science/article/pii/S0009898117300256?via%3Dihub
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