The urinary
bile acid profile, obtained by capillary gas chromatography, of a patient suffering from
cerebrotendinous xanthomatosis and treated with
ursodeoxycholic acid demonstrated, besides the occurrence of 23-norcholic
acid and (23R)-hydroxycholic
acid (as a consequence of this disease), six additional unknown
bile acids and three known
bile acids, viz.
ursodeoxycholic acid,
hyocholic acid and
omega-muricholic acid. The structure of two of the unknown
bile acids were elucidated and proven by organic syntheses. These were 23-norursodeoxycholic
acid and 3 beta-
ursodeoxycholic acid. The structures of three
bile acids were tentatively elucidated as being 1 beta-hydroxyursodeoxycholic
acid, 21-hydroxyursodeoxycholic
acid and 22-hydroxyursodeoxycholic
acid, and the possibility that the structure of the remaining
bile acid is that of 5-hydroxyursodeoxycholic
acid is discussed. Two of these
bile acids (1 beta-hydroxyursodeoxycholic
acid and 5-hydroxyursodeoxycholic
acid) also occurred in urine of a healthy individual during oral
ursodeoxycholic acid treatment, whereas 23-norcholic
acid, 23-norursodeoxycholic
acid, (23R)-hydroxycholic
acid, 21-hydroxyursodeoxycholic
acid and 22-hydroxyursodeoxycholic
acid were only present in urine of the patient suffering from
cerebrotendinous xanthomatosis. The metabolism of
ursodeoxycholic acid, both in the normal state and in the
cerebrotendinous xanthomatosis, is discussed.