title: Analysis of fatty acids and sphingosines from urinary sulfatides in a patient with metachromatic leukodystrophy by gas chromatography-mass spectrometry.
abstract: The urinary sulfatides in metachromatic leukodystrophy (MLD) were analyzed by gas chromatography-mass spectrometry. Fatty acids and long chain bases were obtained after methanolysis. C22:0 and C22h:0 were major components of the fatty acids distributed in the urinary sulfatides in MLD while they were only minor components of the fatty acids in the brain sulfatides in a control subject. These results were in accordance with the report of Philippart et al. It was suggested that the urinary sulfatides originated not in the brain but in other organs. The mass spectra of the trimethylsilyl derivatives of the hydroxy fatty acid methyl esters always showed peaks at m/z (M-15-28)+ and (M-59)+, indicating that the hydroxy group was on carbon 2. Two kinds of long chain base were identified: C18-sphingosine and 3-O-methyl-C18-sphingosine. The latter compound may be a by-product formed on methanolysis.
mesh_terms: D001923:Brain Chemistry; D055598:Chemical Phenomena; D002621:Chemistry; D002675:Child, Preschool; D002849:Chromatography, Gas; D005227:Fatty Acids / Q000032:analysis; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000178:diet therapy / Q000652:urine*; D008297:Male; D013058:Mass Spectrometry; D013110:Sphingosine / Q000032:analysis; D013433:Sulfoglycosphingolipids / Q000652:urine*; D014801:Vitamin A / Q000008:administration & dosage
chemical_list: D005227:Fatty Acids; D013433:Sulfoglycosphingolipids; D014801:Vitamin A; D013110:Sphingosine