J Lipid Res. 1990 Oct;31(10):1895-902.

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

Kuramoto T, Furukawa Y, Nishina T, Sugimoto T, Mahara R, Tohma M, Kihira K, Hoshita T.

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Institute of Pharaceutical Sciences, Hiroshima University School of Medicine, Japan.

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.

PMID:: 2079611; [PubMed - indexed for MEDLINE]

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Bile Acids and Salts

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