Search Results - "Wisse, Patrick"
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Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases
Published in FEBS letters (01-03-2016)“…Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity of glycosphingolipid catabolizing glycosidases. We…”
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Gaucher disease and Fabry disease: New markers and insights in pathophysiology for two distinct glycosphingolipidoses
Published in Biochimica et biophysica acta (01-05-2014)“…Gaucher disease (GD) and Fabry disease (FD) are two relatively common inherited glycosphingolipidoses caused by deficiencies in the lysosomal glycosidases…”
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Glucosylated cholesterol in mammalian cells and tissues: formation and degradation by multiple cellular β-glucosidases[S]
Published in Journal of lipid research (01-03-2016)“…The membrane lipid glucosylceramide (GlcCer) is continuously formed and degraded. Cells express two GlcCer-degrading β-glucosidases, glucocerebrosidase (GBA)…”
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Mass spectrometric quantification of glucosylsphingosine in plasma and urine of type 1 Gaucher patients using an isotope standard
Published in Blood cells, molecules, & diseases (01-04-2015)“…Deficiency of glucocerebrosidase (GBA) leads to Gaucher disease (GD), an inherited disorder characterised by storage of glucosylceramide (GlcCer) in lysosomes…”
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Near-infrared labeled, ovalbumin loaded polymeric nanoparticles based on a hydrophilic polyester as model vaccine: In vivo tracking and evaluation of antigen-specific CD8+ T cell immune response
Published in Biomaterials (01-01-2015)“…Abstract Particulate antigen delivery systems aimed at the induction of antigen-specific T cells form a promising approach in immunotherapy to replace…”
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Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patients
Published in Orphanet journal of rare diseases (24-03-2016)“…We retrospectively compared biochemical responses in type 1 Gaucher disease patients to treatment with glycosphingolipid synthesis inhibitors miglustat and…”
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Lyso-glycosphingolipid abnormalities in different murine models of lysosomal storage disorders
Published in Molecular genetics and metabolism (01-02-2016)“…In lysosomal glycosphingolipid storage disorders, marked elevations in corresponding glycosphingoid bases (lyso-glycosphingolipids) have been reported, such as…”
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Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with (13)C-encoded natural S1P as internal standard
Published in Clinica chimica acta (01-08-2016)“…We developed a mass spectrometric procedure to quantify sphingosine-1-phosphate (S1P) in biological materials. The use of newly synthesized (13)C5 C18-S1P and…”
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Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with 13C-encoded natural S1P as internal standard
Published in Clinica chimica acta (01-08-2016)“…We developed a mass spectrometric procedure to quantify sphingosine-1-phosphate (S1P) in biological materials. The use of newly synthesized 13C5 C18-S1P and…”
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Synthesis of a Panel of Carbon-13-Labelled (Glyco)Sphingolipids
Published in European journal of organic chemistry (01-04-2015)“…The synthesis of a focussed library of sphingolipids differing in the number and position of 13C labels is described. The synthesised sphingolipids differ in…”
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Synthesis of 6‑Hydroxysphingosine and α‑Hydroxy Ceramide Using a Cross-Metathesis Strategy
Published in Journal of organic chemistry (17-07-2015)“…In this paper, a new synthetic route toward 6-hydroxysphingosine and α-hydroxy ceramide is described. The synthesis employs a cross-metathesis to unite a…”
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