Synthetic sulfogalactosylceramide (sulfatide) and its use for the mass spectrometric quantitative urinary determination in metachromatic leukodystrophies.
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.
Cui
Yanli
Y
Département de Chimie, Ecole Normale Supérieure, UMR 8642, CNRS-ENS-UPMC, 24 rue Lhomond, 75231 Paris cedex 05, France.
Colsch
Benoit
B
Afonso
Carlos
C
Alonso
Carlos
C
Baumann
Nicole
N
Tabet
Jean-Claude
JC
Mallet
Jean-Maurice
JM
Zhang
Yongmin
Y
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
C529954:3-O-sulfogalactosyl-1'-1-N-heptadecanoylsphingosine; D013433:Sulfoglycosphingolipids; D002553:Cerebroside-Sulfatase
J Physiol Paris. 2002 Apr-Jun;96(3-4):301-6
12445909
Mol Genet Metab. 2001 May;73(1):30-7
11350180
Neurology. 2005 May 10;64(9):1632-4
15883332
J Neuroimmunol. 2003 Jun;139(1-2):76-80
12799023
Carbohydr Res. 2004 Mar 15;339(4):885-90
14980833
APMIS. 2003 Sep;111(9):867-77
14510644
Prog Lipid Res. 1997 Dec;36(4):245-319
9640458
Autoimmunity. 2002 Nov;35(7):463-8
12685874
Mol Genet Metab. 2003 Jun;79(2):83-90
12809637
J Neurosci Res. 2002 Jul 1;69(1):86-93
12111819
Biochem Biophys Res Commun. 1993 Jul 15;194(1):266-73
8333841
Carbohydr Res. 1994 Jun 17;259(2):201-18
8050096
Hum Mol Genet. 2005 May 1;14(9):1139-52
15772092
Bioorg Med Chem. 1994 Nov;2(11):1143-51
7538868
Anal Biochem. 1999 May 1;269(2):304-11
10222002
J Biol Chem. 2002 Jun 7;277(23):20386-98
11919180
Neurology. 2006 Sep 12;67(5):859-63
16966551
Biochim Biophys Acta. 1998 Jun 15;1392(2-3):202-16
9630631
J Med Chem. 1997 Sep 26;40(20):3234-47
9379443
Carbohydr Res. 1986 Aug 1;150:199-212
3756957
J Lipid Res. 2004 Feb;45(2):281-6
14595000
Clin Biochem. 1997 Jun;30(4):325-31
9209791
Nat Med. 2006 Jan;12(1):138-43
16341241