To study the role of C25-HYDROXY BILE ALCOHOLS AS PRECURSORS OF CHOlic acid, [G-3-H]5beta-cholestane-3alpha,7alpha12alpha,25-tetrol was administered intravenously to two subjects with cerebrotendinous xanthomatosis (CTX) and two normal individuals. One day after pulse labeling, radioactivity was present in the cholic acid isolated from the bile and feces of the subjects with CTX and the bile of the normal individuals. In the two normal subjects, the sp act decay curves of [G-3-H]-cholic acid were exponential, and no traces of [G-3-H]-5beta-cholestane-3alpha,7alpha,12alpha,25-tetrol were detected. In contrast, appreciable quantities of labeled 5beta-cholestane-3alpha,-7aopha,12alpha,25-tetrol were present in the bile and feces of the CTX subjects. The sp act vs. time curves of fecal [G-3-H]5beta-cholestane-3alpha,7alpha,12alpha,25-tetrol and [G-3-H]-cholic acid showed a precursor-product relationship. Although these results suggest that 5beta-cholestane-3alpha,7alpha,12alpha,25-tetrol may be a precursor of cholic acid in man, the possibility that C26-hydroxy intermediates represent the normal pathway can not be excluded.
G Salen, S Shefer, T Setoguchi, E H Mosbach
Title and authors | Publication | Year |
---|---|---|
Synthesis of TUDCA from chicken bile: immobilized dual-enzymatic system for producing artificial bear bile substitute
Shijing T, Yinping P, Qiong Y, Deshuai L, Liancai Z, Jun T, Shaoyong L, Bochu W |
Microbial Cell Factories | 2024 |
Inborn Errors of Metabolism that Lead to Permanent Liver Injury
FK Ghishan |
Zakim and Boyer s Hepatology | 2012 |
Advances in Clinical Chemistry
M Mahler |
Advances in Clinical Chemistry Volume 54 | 2011 |
Clinical Hepatology
H Dancygier |
2010 | |
Elevated cholesterol precursors other than cholestanol can also be a hallmark for CTX
MG de Sain-van der Velden, A Verrips, BH Prinsen, M de Barse, R Berger, G Visser |
Journal of Inherited Metabolic Disease | 2008 |
Two novel mutations in the sterol 27-hydroxylase gene causing cerebrotendinous xanthomatosis
S Lamon-Fava, EJ Schaefer, R Garuti, G Salen, S Calandra |
Clinical Genetics | 2002 |
Two novel mutations in the sterol 27-hydroxylase gene causing cerebrotendinous xanthomatosis: Two novel mutations of CYP27 causing CTX
S Lamon-Fava, EJ Schaefer, R Garuti, G Salen, S Calandra |
Clinical Genetics | 2002 |
Urinary bile alcohol profiles in healthy and cholestatic children
M Nakagawa, M Une, S Takenaka, Y Tazawa, S Nozaki, T Imanaka, T Kuramoto |
Clinica Chimica Acta | 2001 |
Urinary bile alcohol profile in infants with intrahepatic cholestasis: identification of 5β-cholestane-3α,7α,24,25-tetrol
M Nakagawa, M Une, S Takenaka, T Kuramoto, D Abukawa, K Iinuma |
Acta Paediatrica | 1999 |
Identification of (23S)-5α-cholestane-3α,7α,12α,23,25-pentol in urine of patients with cerebrotendinous xanthomatosis
K Kenji, F Katsutoshi, K Taiju, K Masaru, F Jiro, O Mitsuhiro, H Takahiko |
Steroids | 1991 |
The Bile Acids: Chemistry, Physiology, and Metabolism
KD Setchell, D Kritchevsky, PP Nair |
1988 | |
High pressure liquid chromatography solvent systems for studies of bile acid biosynthesis
K Prydz, BF Kase, JI Pedersen |
Journal of lipid research | 1988 |
Contraceptive steroids alter the steady-state kinetics of bile acids
GT Everson, P Fennessey, F Kern |
Journal of lipid research | 1988 |
Cerebrotendinous xanthomatosis: A review of biochemical findings of the patient population in the netherlands
BJ Koopman, BG Wolthers, JC van der Molen, W van der Slik, RJ Waterreus, A van Spreeken |
Journal of Inherited Metabolic Disease | 1988 |
Separation of methylated free bile acids from their taurine and methyl glycine conjugates by thin-layer chromatography
MJ Bolt |
Journal of lipid research | 1987 |
Demonstration of 26-hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosis
S Skrede, I Björkhem, EA Kvittingen, MS Buchmann, SO Lie, C East, S Grundy |
Journal of Clinical Investigation | 1986 |
Increased (23R)-hydroxylase activity in patients suffering from cerebrotendinous xanthomatosis, resulting in (23R)-hydroxylation of bile acids
BJ Koopman, BG Wolthers, JC der Molen, GT Nagel, H Rutgers, B Strijtveen, B Kaptein |
Biochimica et Biophysica Acta (BBA) - General Subjects | 1986 |
HepG2. A human hepatoblastoma cell line exhibiting defects in bile acid synthesis and conjugation
GT Everson, MA Polokoff |
The Journal of biological chemistry | 1986 |
Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26
G Salen, S Shefer, GS Tint, G Nicolau, B Dayal, AK Batta |
Journal of Clinical Investigation | 1985 |
Formation and Metabolism of Bile Alcohols in Man
G Karlaganis, J SjÖVall |
Hepatology | 1984 |
Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study
I Björkhem, O Fausa, G Hopen, H Oftebro, JI Pedersen, S Skrede |
Journal of Clinical Investigation | 1983 |
Diagnosis of cerebrotendinous xanthomatosis (CTX) and effect of chenodeoxycholic acid therapy by analysis of urine using capillary gas chromatography
BG Wolthers, M Volmer, J der Molen, BJ Koopman, AE Jager, RJ Waterreus |
Clinica Chimica Acta | 1983 |
New Comprehensive Biochemistry
S Yamamoto |
New Comprehensive Biochemistry | 1983 |
Synthesis of 3 alpha,7 alpha,12 alpha,25-tetrahydroxy-5 beta-cholestan-24-one, an intermediate in the 25-hydroxylation pathway of cholic acid biosynthesis from cholesterol
B Dayal, GS Tint, AK Batta, S Shefer, G Salen, AK Bose, BN Pramanik |
Journal of lipid research | 1983 |
Bile Acid Metabolism in Cirrhosis. VIII. Quantitative Evaluation of Bile Acid Synthesis From [7β-3H]7α-Hydroxycholesterol and [G-3H]26-Hydroxycholesterol
M Goldman, ZR Vlahcevic, CC Schwartz, J Gustafsson, L Swell |
Hepatology | 1982 |
Serum bile acid profiles in cerebrotendinous xanthomatosis
T Beppu, Y Seyama, T Kasama, S Serizawa, T Yamakawa |
Clinica Chimica Acta | 1982 |
Chemical lonization-mass spectrometric approach to structure determination of an intermediate in bile acid biosynthesis
S Shefer, G Salen, FW Cheng, B Dayal, AK Batta, GS Tint, AK Bose, BN Pramanik |
Analytical Biochemistry | 1982 |
A quantitative evaluation of the conversion of 25-hydroxycholesterol to bile acids in man
L Swell, CC Schwartz, J Gustafsson, H Danielsson, ZR Vlahcevic |
Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism | 1981 |
Cerebrotendinous xanthomatosis: defective liver mitochondrial hydroxylation of chenodeoxycholic acid precursors
H Oftebro, I Björkhem, FC Størmer, JI Pedersen |
Journal of lipid research | 1981 |
Assay of intermediates in bile acid biosynthesis using isotope dilution–mass spectrometry: hepatic levels in the normal state and in cerebrotendinous xanthomatosis
I Björkhem, H Oftebro, S Skrede, JI Pedersen |
Journal of lipid research | 1981 |
Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid
H Oftebro, I Björkhem, S Skrede, A Schreiner, JI Pederson |
Journal of Clinical Investigation | 1980 |
Bile acid metabolism in cirrhosis
TE Patteson, ZR Vlahcevic, CC Schwartz, J Gustafsson, H Danielsson, L Swell |
Gastroenterology | 1980 |
An in vivo evaluation of the quantitative significance of several potential pathways to cholic and chenodeoxycholic acids from cholesterol in man
L Swell, J Gustafsson, CC Schwartz, LG Halloran, H Danielsson, ZR Vlahcevic |
Journal of lipid research | 1980 |
Biosynthesis of bile acids in man. Multiple pathways to cholic acid and chenodeoxycholic acid
ZR Vlahcevic, CC Schwartz, J Gustafsson, LG Halloran, H Danielsson, L Swell |
The Journal of biological chemistry | 1980 |
Biliary bile acids, bile alcohols, and sterols of Alligator mississippiensis
GS Tint, B Dayal, AK Batta, S Shefer, T Joanen, L McNease, G Salen |
Journal of lipid research | 1980 |
Occurrence of bile alcohols in the bile of a patient with cholestasis
A Kibe, S Nakai, T Kuramoto, T Hoshita |
Journal of lipid research | 1980 |
Cholic Acid Biosynthesis THE ENZYMATIC DEFECT IN CEREBROTENDINOUS XANTHOMATOSIS
G Salen, S Shefer, FW Cheng, B Dayal, AK Batta, GS Tint |
Journal of Clinical Investigation | 1979 |
Preparation of [3β-3H] labeled bile acids and bile alcohols
B Dayal, E Bagan, GS Tint, S Shefer, G Salen |
Steroids | 1979 |
Formation of cholic acid via 3α,7α,12α-trihydroxy-5β-cholestan-26-oic acid in the dog
LE Kajer, GC Williams, P Szczepanik, S Silvis, RF Hanson |
Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism | 1979 |
C26-analogs of naturally occurring bile alcohols-II. Preparation of 24-nor-5β-cholestane-3α,7α,12α,23-tetrols (23R and 23S) and 24-nor-5β-cholestane-3α,7α,12α,26-tetrols (25R and 25S) by a hydroboration procedure
B Dayal, GS Tint, G Salen |
Steroids | 1979 |
Defects of bile acid synthesis in Zellweger's syndrome
RF Hanson, P Szczepanik-VanLeeuwen, GC Williams, G Grabowski, HL Sharp |
Science | 1979 |
Configurational assignment of 5β-cholestane-3α, 7α, 12α, 23, 25-pentol excreted by patients with cerebrotendinous xanthomatosis (a circular dichroism study)
B Dayal, GS Tint, S Shefer, G Salen |
Steroids | 1979 |
Metabolism of potential precursors of chenodeoxycholic acid in cerebrotendinous xanthomatosis (CTX)
G Salen, S Shefer, EH Mosbach, S Hauser, BI Cohen, G Nicolau |
Journal of lipid research | 1979 |
Metabolism of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 26-tetrol and 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25-tetrol into cholic acid in normal human subjects
RF Hanson, AB Staples, GC Williams |
Journal of lipid research | 1979 |
Hepatic cholesterol metabolism in normo- and hyperlipidemic patients with cholesterol gallstones
J Ahlberg, B Angelin, I Björkhem, K Einarsson, B Leijd |
Journal of lipid research | 1979 |
Effect of taurocholate on the conversion of 3α, 7α, 12α-Trihydroxy-5β-Cholestan-26-OIC acid into cholic acid
RF Hanson, GC Williams |
Steroids | 1978 |
Synthesis of biological precursors of cholic acid
B Dayal, AK Batta, S Shefer, GS Tint, G Salen |
Steroids | 1978 |
Intrahepatic pigment and crystal forms in patients with cerebrotendinous xanthomatosis (CTX)
G Salen, FG Zaki, S Sabesin, D Boehme, S Shefer, EH Mosbach |
Gastroenterology | 1978 |
Comparative effects of ursodeoxycholic acid and chenodeoxycholic acid in the rhesus monkey
T Fedorowski, G Salen, FG Zaki, S Shefer, EH Mosbach |
Gastroenterology | 1978 |
Synthesis and Enterohepatic Circulation of Bile Salts
RF Hanson, JM Pries |
Gastroenterology | 1977 |
Metabolism of Ursodeoxycholic Acid in Man
T Fedorowski, G Salen, A Colallilo, GS Tint, EH Mosbach, JC Hall |
Gastroenterology | 1977 |
Inborn Errors of Bile Acid Synthesis
G Salen, S Shefer, F Zaki, E Mosbach |
1977 | |
Cholic acid biosynthesis: conversion of 5β-cholestane-3α,7α,12α,25-tetrol into 5β-cholestane-3α,7α, 12α,24β,25-pentol by human and rat liver microsomes
FW Cheng, S Shefer, B Dayal, GS Tint, T Setoguchi, G Salen, EH Mosbach |
Journal of lipid research | 1977 |
Metabolism of 3α, 7α, 12α-trihydroxy-5β-cholestan-26-oic acid in normal subjects with an intact enterohepatic circulation
RF Hanson, GC Williams |
Journal of lipid research | 1977 |
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat
S Shefer, FW Cheng, B Dayal, S Hauser, GS Tint, G Salen, EH Mosbach |
Journal of Clinical Investigation | 1976 |
The Hepatobiliary System
W Taylor |
1976 | |
Quantitative aspects of the conversion of 5 beta-cholestane intermediates to bile acids in man
CC Schwartz, BI Cohen, ZR Vlahcevic, DH Gregory, LG Halloran, T Kuramoto, EH Mosbach, L Swell |
The Journal of biological chemistry | 1976 |
Metabolism of bile alcohols in the perfused rabbit liver
BI Cohen, T Kuramoto, MA Rothschild, EH Mosbach |
The Journal of biological chemistry | 1976 |
Side chain hydroxylations in biosynthesis of cholic acid. 25- and 26-Hydroxylation of 5beta-cholestane-3alpha, 7alpha, 12alpha-triol by reconstituted systems from rat liver microsomes
I Björkhem, H Danielsson, K Wikvall |
The Journal of biological chemistry | 1976 |
Chenodeoxycholic acid inhibits increased cholesterol and cholestanol synthesis in patients with cerebrotendinous Xanthomatosis
G Salen, TW Meriwether, G Nicolau |
Biochemical Medicine | 1975 |