title: 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.affiliation: Institute of Pharaceutical Sciences, Hiroshima University School of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kuramoto
authors.forename: Y
authors.initials: Y
authors.lastname: Furukawa
authors.forename: T
authors.initials: T
authors.lastname: Nishina
authors.forename: T
authors.initials: T
authors.lastname: Sugimoto
authors.forename: R
authors.initials: R
authors.lastname: Mahara
authors.forename: M
authors.initials: M
authors.lastname: Tohma
authors.forename: K
authors.initials: K
authors.lastname: Kihira
authors.forename: T
authors.initials: T
authors.lastname: Hoshita
mesh_terms: D001647:Bile Acids and Salts / Q000652:urine*; D001927:Brain Diseases / Q000652:urine*; D002850:Chromatography, Gel; D006801:Humans; D015394:Molecular Structure; D014973:Xanthomatosis / Q000652:urine*
chemical_list: D001647:Bile Acids and Salts