Identification of glucosyl sphingosine from Gaucher's spleen by gas chromatography-electron impact and GC-chemical ionization mass spectrometry.
Glucosyl sphingosine was isolated from the spleen of a patient with adult-type Gaucher's disease. The yield of purified glucosyl sphingosine was 9.3 nmoles/g wet tissue. The gas chromatography-chemical ionization mass spectrum of acetylated glucosyl sphingosine showed peaks due to the presence of ions formed by successive loss of acetic acid (mass 60) from the molecular ion (QM+, 714). Fragments from acetylated sugar, m/e 331, and from the sphingosine residue, m/e 306, were also detected. The GC-CI mass spectrum of the trimethylsilyl derivative of glucosyl sphingosine showed peaks due to the molecular ion (QM+, 822), ions from the sugar moiety (m/e 361, 271), and ions from the sphingosine base (m/e 264, 280). Fragmentation analysis of the purified sample by GC-EI and GC-CI mass spectrometry confirmed the structure glucopyranosyl(1 leads to 1)-1,3-dihydroxy-2-amino-4-octadecene.
D000328:Adult; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D005776:Gaucher Disease / Q000378:metabolism*; D005960:Glucosides / Q000032:analysis*; D006027:Glycosides / Q000032:analysis*; D006801:Humans; D013110:Sphingosine / Q000031:analogs & derivatives* / Q000032:analysis; D013154:Spleen / Q000378:metabolism*
D005960:Glucosides; D006027:Glycosides; D013110:Sphingosine