PMID: 3756224Oct 1, 1986Paper

Increased (23R)-hydroxylase activity in patients suffering from cerebrotendinous xanthomatosis, resulting in (23R)-hydroxylation of bile acids

Biochimica Et Biophysica Acta
B J KoopmanB Kaptein

Abstract

Patients suffering from cerebrotendinous xanthomatosis, an inborn error of metabolism in bile acid synthesis, excrete excessive amounts of 23-hydroxylated bile alcohols, 23-norcholic acid and 23-hydroxycholic acid into urine. In this study the configuration of this excreted 23-hydroxycholic acid was established as (23R)-hydroxycholic acid. Urine samples of two treated patients, receiving chenodeoxycholic acid, were investigated to see whether this administered bile acid was partly converted into 23-hydroxychenodeoxycholic acid. One patient was treated with ursodeoxycholic acid for 1 month and subsequently with chenodeoxycholic acid, and the urinary excretion of both (23R)-hydroxychenodeoxycholic acid and (23R)-hydroxyursodeoxycholic acid were followed. Indeed, all three patients excreted (23R)-hydroxylated chenodeoxycholic acid during oral treatment with chenodeoxycholic acid, and the patient treated with ursodeoxycholic acid excreted (23R)-hydroxylated ursodeoxycholic acid. During treatment with chenodeoxycholic acid the excretion of (23R)-hydroxychenodeoxycholic acid increases at first and later on decreases markedly. These findings suggest increased (23R)-hydroxylase activity in patients suffering from cerebrotendinous xanth...Continue Reading

References

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Citations

Jan 1, 1988·Journal of Inherited Metabolic Disease·B J KoopmanA van Spreeken
Jan 1, 1989·Comparative Biochemistry and Physiology. B, Comparative Biochemistry·M JirsaK Kucera
Jan 1, 1987·Clinical Neurology and Neurosurgery·R J WaterreusH J Oosterhuis
Oct 1, 1994·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·H U MarschallS Matern

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