Determination of Urinary Sulfatides and Other Lipids by Combination of Reversed-Phase and Thin-Layer Chromatographies

Berná, Linda; Asfaw, Befekadu; Conzelmann, Ernst; Černý, Bohuslav; Ledvinová, Jana

doi:10.1006/abio.1999.4002

Cited by 32 publications

(24 citation statements)

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“…PC is one of the most abundant cell membrane lipids; therefore, normalizing the urinary samples for total PC will effectively correct for the amount of urinary sediment. We have used PC to correct for the amount of urinary sediment in earlier studies (16 ), and SM has also been proposed for this purpose (17 ). However, because SM concentrations are potentially altered through the aberrations of lipid trafficking observed in lysosomal storage disorders (14 ), we have continued to use PC as a representative cellular lipid to correct for the amounts of urinary sediment.…”

Section: Discussionmentioning

confidence: 99%

Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes

et al. 2005

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Background: Fabry disease is an X-linked lysosomal storage disorder resulting from a deficiency of the lysosomal hydrolase, ␣-galactosidase, for which enzyme replacement therapy is now available. In this study, we aimed to identify Fabry heterozygotes not only for genetic counseling of families but because it is becoming increasingly obvious that many heterozygous (carrier) females are symptomatic and should be considered for treatment. Methods: We measured 29 individual lipid species, including ceramide, glucosylceramide, lactosylceramide, and ceramide trihexoside, in urine samples from Fabry hemizygotes and heterozygotes and from control individuals by electrospray ionization tandem mass spectrometry. Individual analyte species and analyte ratios were analyzed for their ability to differentiate the control and patient groups. Results: The Fabry hemizygotes had increased concentrations of the substrate for the deficient enzyme, ceramide trihexoside, as well as lactosylceramide and ceramide, along with decreased concentrations of both glucosylceramide and sphingomyelin. Ratios of these analytes improved differentiation between the control and Fabry groups, with the Fabry heterozygotes generally falling between the Fabry hemizygotes and the control group. Conclusions: These lipid profiles hold particular promise for the identification of Fabry individuals, may aid

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Section: Discussionmentioning

confidence: 99%

Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes

et al. 2005

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“…Several methods have been used to quantify sulfatides, including TLC ( 19 ), HPLC ( 14,20 ), and, more recently, MS ( 4,13,15,(21)(22)(23)(24)(25). Here we describe a LC/MS/MS method to quantify sulfatides and their molecular species over a wide range of concentrations in normal and diseased tissues, plasma, and urine.…”

Section: Extraction Of Sulfatides From Plasma and Tissue Homogenatesmentioning

confidence: 99%

“…In contrast to recently published methods on the quantifi cation of sulfatides in dried blood spots and urine ( 24,25 ) from MLD patients and normal controls, our method could easily quantify sulfatides in normal plasma and urine with a signal to noise ratio of >10 for all peaks quantifi ed. Because of its sensitivity, only 0.5 ml of a representative urine sample suffi ces for the analysis of MLD and normal urines instead of the large volumes or even 24 h urine collections, which are needed when traditional methods like TLC or HPLC are used ( 14,19,32 ).…”

Section: Measurement Of Different Molecular Sulfatide Species In Fl Umentioning

confidence: 99%

Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometry

Mirzaian

Kramer²,

Poorthuis

2015

Journal of Lipid Research

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This article is available online at http://www.jlr.org high concentrations are found in selected organs, including kidney, colon, pancreas, and gall bladder. Increased concentrations of sulfatides have been reported in renal cell carcinoma, adenocarcinoma of colon and lung, and ovarian cancer ( 2, 4 ). Sulfatides have been proposed to play a role as ion barriers to osmotic stress and as counterions of ammonium in the renal adaptation to chronic metabolic acidosis ( 5,6 ) Sulfatides are synthesized from their precursor galactosylceramide in the Golgi apparatus by 3-O-sulfation of the galactose residue by the enzyme 3 ′ -phosphoadenosine-5 ′ -phosphosulfate: cerebroside sulfotransferase (CST) (EC2.8.2.11). Galactosylceramide is synthesized in the endoplasmic reticulum from ceramides and UDP-galactose by the enzyme UDP-galactose:ceramide galactosyltransferase (CGT) (EC 2.4.1.45) and is then transported to the Golgi apparatus prior to sulfation to sulfatides. Sulfatides consist of many molecular species, with structures differing in acyl chain length and hydroxylation and sphingoid base. These sulfatide molecular species are cell and tissue specifi c, which may be explained by the cell-and tissuespecifi c expression of ceramide synthases and fatty acid 2-hydroxylase ( 1, 7 ). It is diffi cult to assign specifi c functions to the different molecular species of sulfatides, but sulfatides with C16:0 fatty acids were reported to be involved in the regulation of insulin secretion in rat pancreatic ␤ -cells ( 8 ), and elevated levels of C18:0 sulfatides have been implicated as a cause of audiogenic seizers in mice overexpressing CGT ( 9 ). There is also heterogeneity of the sphingoid base composition of sulfatides. Most of the Abstract Sulfatides are found in brain as components of myelin, oligodendrocytes, and neurons but are also present in various visceral tissues. Metachromatic leukodystrophy (MLD) is an inherited lysosomal storage disorder caused by a defi ciency of arylsulfatase A, leading to severe white matter disease due to the accumulation of sulfatides and lysosulfatides. To study the physiological role of sulfatides, accessible and sensitive quantitative methods are required. We developed a sensitive LC/MS/MS method to quantify total sulfatide and lysosulfatide content as well as individual molecular species in urine and plasma from MLD patients and plasma and tissues from an MLD mouse model. Our results demonstrate that the method can quantify a wide range of sulfatide concentrations and can be used to quantify total sulfatide content and levels of individual molecular species of sulfatides in tissues, cells, and body fl uids. Even though plasma sulfatides and lysosulfatides would seem attractive candidate biomarkers that could possibly correlate with the severity of MLD and be of use to monitor the effects of therapeutic intervention, our results indicate that it is unlikely that the determination of these storage products in plasma will be useful in this respect. Sulfatides are anionic sulfoglycolipids main...

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“…8B). It is also possible to use the ratio of the analyzed compound to sphingomyelin (Berna, et al, 1999;Kuchar, et al, 2009) or phosphatidylcholine (Fuller, et al, 2005;Whitfield, et al, 2001), which are membrane-bound lipids that can be measured simultaneously in the same sample. …”

Section: Standardization Of Quantitative Data In Urinementioning

confidence: 99%

Tandem Mass Spectrometry of Sphingolipids: Application in Metabolic Studies and Diagnosis of Inherited Disorders of Sphingolipid Metabolism

Kuchař¹,

Asfaw²,

Ledvinová³

2012

Tandem Mass Spectrometry - Applications and Principles

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Determination of Urinary Sulfatides and Other Lipids by Combination of Reversed-Phase and Thin-Layer Chromatographies

Cited by 32 publications

References 31 publications

Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes

Urinary Lipid Profiling for the Identification of Fabry Hemizygotes and Heterozygotes

Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometry

Tandem Mass Spectrometry of Sphingolipids: Application in Metabolic Studies and Diagnosis of Inherited Disorders of Sphingolipid Metabolism

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