Identification of short side chain bile acids in urine of patients with cerebrotendinous xanthomatosis.

Abstract

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.

• Authors: T Kuramoto, Y Furukawa, T Nishina, T Sugimoto, R Mahara, M Tohma, K Kihira, T Hoshita
• Journal: Journal of lipid research (J Lipid Res) Vol. 31 Issue 10 Pg. 1895-902 (Oct 1990) ISSN: 0022-2275 [Print] United States
• PMID: 2079611 (Publication Type: Journal Article)

Chemical References

• Bile Acids and Salts

Topics

• Bile Acids and Salts (urine)
• Brain Diseases (urine)
• Chromatography, Gel
• Humans
• Molecular Structure
• Xanthomatosis (urine)