Bile acids and conjugates identified in metabolic disorders by fast atom bombardment and tandem mass spectrometry.
From a study of the collision-activated fragmentation of bile acids, a qualitative analytical method based on negative ion fast atom bombardment tandem mass spectrometry has been developed. The times for sample preparation and analyses are short. Both free and conjugated bile acids are detected as they occur in biological fluids, without derivatization. For identifying bile acids and conjugates, the method offers better specificity and sensitivity than does the fast atom bombardment mass spectrometric technique alone. Specific scan modes have been developed for the selective detection of taurine conjugates, delta 4-unsaturated taurine conjugates, delta 4-3-keto free acids and their glycine conjugates, free acids and glycine conjugates bearing a hydroxyl group at the C-12 position, sulfates of glycine and taurine conjugates, and a C29 dicarboxylic bile acid, specific for generalized peroxisomal disorders. Applications of this technique demonstrate its potential usefulness, principally in the diagnosis of several peroxisomal disorders.
Libert
R
R
Department of Neuropediatry, Cliniques Universitaires St. Luc, Brussels, Belgium.
Hermans
D
D
Draye
J P
JP
Van Hoof
F
F
Sokal
E
E
de Hoffmann
E
E
D000326:Adrenoleukodystrophy / Q000378:metabolism; D001647:Bile Acids and Salts / Q000032:analysis* / Q000097:blood / Q000652:urine; D005998:Glycine / Q000032:analysis / Q000097:blood / Q000652:urine; D006801:Humans; D006900:Hydroxylation; D013058:Mass Spectrometry; D008659:Metabolic Diseases / Q000378:metabolism*; D008830:Microbodies / Q000502:physiology; D012035:Refsum Disease / Q000378:metabolism; D016339:Spectrometry, Mass, Fast Atom Bombardment; D013431:Sulfates / Q000097:blood / Q000378:metabolism / Q000652:urine; D013654:Taurine / Q000032:analysis / Q000097:blood / Q000652:urine; D015211:Zellweger Syndrome / Q000378:metabolism
D001647:Bile Acids and Salts; D013431:Sulfates; D013654:Taurine; D005998:Glycine