Journal of Lipid Research, Vol 31, 1895-1902, Copyright © 1990 by Lipid Research, Inc.
Identification of short side chain bile acids in urine of patients with cerebrotendinous xanthomatosis
T Kuramoto, Y Furukawa, T Nishina, T Sugimoto, R Mahara, M Tohma, K Kihira and T Hoshita
Institute of Pharaceutical Sciences, Hiroshima University School of Medicine, Japan.
Urine from patients with cerebrotendinous xanthomatosis (CTX) was found to
contain a number of minor bile acids along with three major bile acids,
7-epicholic acid, norcholic acid, and cholic acid. The following minor bile
acids were identified by combined gas-liquid chromatography- mass
spectrometry: 7-ketobisnordeoxycholic acid; 12- ketobisnorchenodeoxycholic
acid; 7-ketonordeoxycholic acid; 12- ketochenodeoxycholic acid;
7-ketodeoxycholic acid; 12- ketochendeoxycholic acid; bisnorcholic acid;
allonorcholic acid; allocholic acid; 1 beta-hydroxybisnorcholic acid; 1
beta- hydroxynorcholic acid; 1 beta-hydroxycholic acid; 2 beta-
hydroxybisnorcholic acid; 2 beta-hydroxy-norcholic acid; 2 beta-
hydroxycholic acid. The presence of C22 and C23 bile acids in urine of the
CTX patients suggests that bile alcohols having a hydroxyl group at C22 or
C23 in the side chain may be further degraded to these bile acids.
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