title: Quantification of sulfatides and lysosulfatides in tissues and body fluids by liquid chromatography-tandem mass spectrometry.
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 deficiency 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 fluids. 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.
authors: Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
authors: Mina
authors: M
authors: Mirzaian
authors: Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
authors: Gertjan
authors: G
authors: Kramer
authors: Department of Medical Biochemistry, Academic Medical Center, Amsterdam, The Netherlands.
authors: Ben J H M
authors: BJ
authors: Poorthuis
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D001774:Blood Chemical Analysis / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D002853:Chromatography, Liquid; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007966:Leukodystrophy, Metachromatic / Q000097:blood / Q000473:pathology / Q000652:urine; D008297:Male; D051379:Mice; D008875:Middle Aged; D011609:Psychosine / Q000031:analogs & derivatives* / Q000097:blood / Q000652:urine; D013433:Sulfoglycosphingolipids / Q000097:blood* / Q000652:urine*; D053719:Tandem Mass Spectrometry; D016482:Urinalysis / Q000379:methods*; D055815:Young Adult
chemical_list: D013433:Sulfoglycosphingolipids; D011609:Psychosine; C019064:psychosine-3'-sulfate ester
keywords: biomarker; lipidomics; metachromatic leukodystrophy
references: Lipids Health Dis. 2011;10:28
references: 21299873
references: Rapid Commun Mass Spectrom. 2014 Mar 30;28(6):587-94
references: 24519821
references: J Lipid Res. 2012 Aug;53(8):1437-50
references: 22619219
references: Clin Chem. 2013 Mar;59(3):547-56
references: 23237761
references: Glycoconj J. 2008 Feb;25(2):147-55
references: 17701343
references: Proc Natl Acad Sci U S A. 2008 Feb 26;105(8):2812-7
references: 18287059
references: Mol Neurobiol. 2008 Apr-Jun;37(2-3):93-103
references: 18465098
references: Methods Mol Biol. 2009;580:285-94
references: 19784606
references: Clin Chim Acta. 2014 Jun 10;433:39-43
references: 24370383
references: Biochim Biophys Acta. 2000 Feb 24;1484(1):59-70
references: 10685031
references: Mol Genet Metab. 2001 May;73(1):30-7
references: 11350180
references: J Lipid Res. 2004 Feb;45(2):281-6
references: 14595000
references: J Lipid Res. 1967 Nov;8(6):621-30
references: 6057492
references: Biochim Biophys Acta. 1979 Mar 29;572(3):423-31
references: 435503
references: Biochim Biophys Acta. 1979 Mar 29;572(3):432-41
references: 435504
references: Biochem Biophys Res Commun. 1989 Mar 15;159(2):605-11
references: 2539117
references: Clin Chem. 1996 Feb;42(2):232-8
references: 8595716
references: Cancer. 1996 Jul 1;78(1):43-7
references: 8646725
references: Proc Natl Acad Sci U S A. 1996 Dec 10;93(25):14821-6
references: 8962139
references: Biochim Biophys Acta. 1998 Jun 15;1392(2-3):202-16
references: 9630631
references: Prog Lipid Res. 1997 Dec;36(4):245-319
references: 9640458
references: Anal Biochem. 1999 May 1;269(2):304-11
references: 10222002
references: Anal Biochem. 1999 Oct 1;274(1):90-7
references: 10527500
references: J Biol Chem. 1957 May;226(1):497-509
references: 13428781
references: Can J Biochem Physiol. 1959 Aug;37(8):911-7
references: 13671378
references: Acta Neuropathol. 2004 Oct;108(4):261-71
references: 15322834
references: Diabetes. 2006 Oct;55(10):2826-34
references: 17003349
references: J Neurosci. 2007 Aug 29;27(35):9482-90
references: 17728461
references: Mol Med. 2007 Sep-Oct;13(9-10):471-9
references: 17660863
references: Comp Biochem Physiol B Biochem Mol Biol. 2008 Jan;149(1):161-7
references: 17905621
references: J Neurochem. 2010 Jan;112(1):282-95
references: 19860853
references: Mol Genet Metab. 2010 Feb;99(2):142-8
references: 19815439
references: Mol Cancer. 2010;9:186
references: 20624317
references: Proc Natl Acad Sci U S A. 2010 Nov 9;107(45):19473-8
references: 20962279
references: Proc Natl Acad Sci U S A. 2013 Jun 11;110(24):9998-10003
references: 23716689
references: Clin Chim Acta. 2013 Oct 21;425:153-9
references: 23838369
references: Blood. 2011 Oct 20;118(16):e118-27
references: 21868580