title: Synthetic sulfogalactosylceramide (sulfatide) and its use for the mass spectrometric quantitative urinary determination in metachromatic leukodystrophies.
abstract: 3-O-Sulfogalactosylceramides (sulfatides) accumulate in the genetic disease metachromatic leukodystrophy which is due to a defect in the catabolic enzyme, arylsulfatase A. Clinical diagnosis is usually confirmed by in vitro enzymatic deficiency of arylsulfatase A activity. The diagnosis may be complicated because of arylsulfatase A pseudo-deficiencies and another cause of MLD, sphingolipid activator B deficiency. As large quantities of sulfatides can be found in the urine in this disease, sulfatiduria appears as an extremely useful test. As recently enzyme replacement is underway, the quantitative determination, using an internal standard, appears particularly useful as a follow-up. Thus a non-physiological sulfatide was synthesized for this purpose, i.e. 3-O-sulfo-beta-D-C17 galactosylceramide (3-O-Sulfo-D: -Galactosyl-beta1'-->1-N-Heptadecanoyl-D-erythro-Sphingosine). It has been prepared through condensation of an azidosphingosine derivative with a protected D-galactopyranosyltrichloroacetimidate. Reduction of the azide was followed by acylation of a C-17 fatty acid. The key step was achieved by selective sulfation of the desired hydroxyl group on the sugar residue of the galactosylceramide using the stannylene methodology to give a 3'-sulfated beta-galactosyl C-17 ceramide.
authors: Département de Chimie, Ecole Normale Supérieure, UMR 8642, CNRS-ENS-UPMC, 24 rue Lhomond, 75231 Paris cedex 05, France.
authors: Yanli
authors: Y
authors: Cui
authors: Benoit
authors: B
authors: Colsch
authors: Carlos
authors: C
authors: Afonso
authors: Carlos
authors: C
authors: Alonso
authors: Nicole
authors: N
authors: Baumann
authors: Jean-Claude
authors: JC
authors: Tabet
authors: Jean-Maurice
authors: JM
authors: Mallet
authors: Yongmin
authors: Y
authors: Zhang
mesh_terms: D000328:Adult; D002553:Cerebroside-Sulfatase / Q000172:deficiency / Q000235:genetics; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000201:enzymology / Q000235:genetics / Q000652:urine*; D012015:Reference Standards; D021241:Spectrometry, Mass, Electrospray Ionization / Q000592:standards; D013433:Sulfoglycosphingolipids / Q000138:chemical synthesis / Q000592:standards / Q000652:urine*; D053719:Tandem Mass Spectrometry / Q000592:standards
chemical_list: C529954:3-O-sulfogalactosyl-1'-1-N-heptadecanoylsphingosine; D013433:Sulfoglycosphingolipids; D002553:Cerebroside-Sulfatase
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