Sphingolipids sulfatides

Sphingolipids (sulfatide, ganglioside GM2, and asialo-GM2 (GA2)) were distinctly visible in hexb / mouse brain samples by using OCN for matrix application. These ion images clearly demonstrate that the OCN system is useful for sample preparation for imaging MALDTMS of lipids. The spatial distribution of sulfide subspecies ST dl8 l/C22 0 m/z 862.6), ST(OH) dl8 l/h22 0 m/z... 

Sphingolipids (ceramids, sphingomyelin, cerebrosides, sulfatides, gangliosides) and lipids with secondary amines produce blue spots on a white backgound (CAUTION benzidine is a carcinogen)... 

Our new method of sphingolipid analysis using high performance liquid chromatography allows us to determine not only their quantities but also their homolog compositions in a small amount of tissue. We now feel that less than 1 mg of fresh brain or nerve tissue is sufficient for complete analysis. The application of this new method for analyzing cerebroside and sulfatide-in plaques of brain from a patient with multiple sclerosis has been recently described (15). 

MALDI has also been applied to quantify biological material.214,215 This approach was first used to investigate serum glycolipids.216 Sulfatides were quantified using MALDI-TOF which proved to be sensitive and reliable. More recently, the levels of sphingolipids from cardiac valves of patients with Fabry disease were quantified similarly.217... 

Sphingolipids are not essential nutrients but are increasingly being recognized as important in nutrition, as was reviewed by Vesper et al. (1999). In mammalian tissues and milk, the sphingolipids include ceramides, sphingomyelins, cerebrosides, gangliosides and sulfatides. 

Sphingolipid Any of a number of lipid molecules that contain sphingosine as part of its molecular structure. These include ceramide, sphingomyelin, cerebrosides, sulfatides, and ganghosides. 

Wenger, D. A., and Inui, K., Studies on the sphingolipid activator protein for the enzymatic hydrolyses of CMj ganglioside and sulfatide. In Molecular Basis of Lysosomal Storage Disorders (J. A. Barranger and R. O. Brady, eds.), pp. 61-78. Academic Press, Orlando, 1984. 

Some years ago, Celmer and Carter2 proposed a rational nomenclature for cerebrosides and phosphatides, but Klenk and coworkers3 kept the old names in grouping the zoo-sphingolipids, which were denoted as (a) cerebrosides, (b) sulfatides, and (c) gangliosides. 

Colsch et al., 2007). Because the diagnosis may be complicated in cases of arylsulfatase A pseudodeficiency and sphingolipid activator protein deficiency, this measurement of sulfatide in the urinary sediment of affected individuals by a rapid, sensitive, and specific mass spectrometric method has been long wanted (Whitfield et al., 2001). Urinary sulfatides are now commonly detected using electrospray ionization-tandem mass spectrometry by means of the precursor ion scan 97. Levels are considerably increased to X20-30 folds as compared to controls which allows the rapid screening of a large number of samples. 

Glycosphingolipids are sphingolipids, such as the cerebrosides, globosides, sulfatides, and gangliosides, that contain carbohydrate moieties (Figure 10.8). Glycosphingolipids are significant components of the membranes of nerves. 

Fig. 33.33. S5mthesis of sphingolipids from ceramide. PhosphochoUne or sugars add to the hydroxymethyl group of ceramide (in blue) to form sphingomyelins, cerebrosides, sulfatides, globosides, and ganghosides. Gal = galactose Glc = gucose GalNAc = iV-acetylgalac-tosamine NANA = A-acetylneuraminic acid.

Fig. 3-44). Careful examination of all organs might reveal the presence of metachromatic granules in the renal tubules, the gall bladder, and the intrahepatic bile ducts. In metachromatic leukodystrophy, the levels of sulfatides increase more (2 to 4 times) in the white than in the grey matter. The increase in sulfatides is associated with an increase in cerebrosides. The concentration of the other lipids is nearly normal. The proportion of sphingolipids containing long-chain fatty acids (18 or more carbons) is decreased in metachromatic leukodystrophy, and the ceramides found in the accumulating lipids contain mainly short-chain fatty acids. The relationship between the lipid accumulation and the demyelinization and the symptoms of leukodystrophy remains to be elucidated.

Sphingolipids have been studied by soft-ionization methods, with FDl-MS, FAB-MS, ESI-MS, and MALDI-MS being the most widely used techniques. FDl-MS was applied for the characterization of glycosphingolipids with up to five saccharide units [213], Both FDI-MS and LSIMS-MS were apphed in the analysis of acidic glycosphingolipids, i.e., carrying asialo or sulfatide units [214]. In both cases, both [M+H]+ and [M+Na] was observed, whereas with the acidic compounds [(M-H+Na)+Na] -ion were observed as well. Similar observations have been made using other condensed-phase ionization methods [215, 216]. 

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