Identification of Glucosyl Sphingosine from Gaucher's Spleen by Gas Chromatography-Electron Impact and GC-Chemical Ionization Mass Spectrometry

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Abstract
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↑1)-1, 3-dihydroxy-2-amino-4-octadecene.