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 (mle 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 spectro-metry confirmed the structure glucopyranosyl(1→1)-l, 3-dihydroxy-2-amino-4-octadecene.
