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Citations to this article

Bile alcohol metabolism in man. Conversion of 5beta-cholestane-3alpha, 7alpha,12alpha, 25-tetrol to cholic acid.
G Salen, … , T Setoguchi, E H Mosbach
G Salen, … , T Setoguchi, E H Mosbach
Published July 1, 1975
Citation Information: J Clin Invest. 1975;56(1):226-231. https://doi.org/10.1172/JCI108071.
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Bile alcohol metabolism in man. Conversion of 5beta-cholestane-3alpha, 7alpha,12alpha, 25-tetrol to cholic acid.

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Abstract

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.

Authors

G Salen, S Shefer, T Setoguchi, E H Mosbach

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Total citations by year

Year: 2024 2012 2011 2010 2008 2002 2001 1999 1991 1988 1987 1986 1985 1984 1983 1982 1981 1980 1979 1978 1977 1976 1975 Total
Citations: 1 1 1 1 1 2 1 1 1 4 1 3 1 1 4 3 3 6 9 4 5 5 1 60
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Citations to this article (60)

Title and authors Publication Year
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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
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Contraceptive steroids alter the steady-state kinetics of bile acids
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Cerebrotendinous xanthomatosis: A review of biochemical findings of the patient population in the netherlands
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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
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HepG2. A human hepatoblastoma cell line exhibiting defects in bile acid synthesis and conjugation
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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
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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
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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
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Metabolism of 3α, 7α, 12α-trihydroxy-5β-cholestan-26-oic acid in normal subjects with an intact enterohepatic circulation
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Journal of lipid research 1977
A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat
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Metabolism of bile alcohols in the perfused rabbit liver
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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

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