Literature DB >> 1141434

Bile alcohol metabolism in man. Conversion of 5beta-cholestane-3alpha, 7alpha,12alpha, 25-tetrol to cholic acid.

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

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.

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Year:  1975        PMID: 1141434      PMCID: PMC436573          DOI: 10.1172/JCI108071

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  9 in total

1.  CONVERSION OF CHOLESTEROL TO TRIHYDROXYCOPROSTANIC ACID AND CHOLIC ACID IN MAN.

Authors:  J B CAREY
Journal:  J Clin Invest       Date:  1964-07       Impact factor: 14.808

2.  Formation of trihydroxycoprostanic acid from cholesterol in man.

Authors:  E STAPLE; J L RABINOWITZ
Journal:  Biochim Biophys Acta       Date:  1962-06-04

Review 3.  Recent advances in molecular pathology. Isotopic analysis of metabolic systems. I.

Authors:  J M Reiner
Journal:  Exp Mol Pathol       Date:  1974-02       Impact factor: 3.362

Review 4.  Hepatic synthesis of bile acids. Biochemical steps and mechanisms of rate control.

Authors:  E H Mosbach
Journal:  Arch Intern Med       Date:  1972-10

5.  Oxidation of 5 beta-cholestane-3alpha, 7alpha, 12alpha-triol by rat liver microsomes.

Authors:  T Cronholm; G Johansson
Journal:  Eur J Biochem       Date:  1970-10

6.  Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism.

Authors:  G Salen
Journal:  Ann Intern Med       Date:  1971-12       Impact factor: 25.391

7.  Biosynthesis of bile acids in man. Hydroxylation of the C27-steroid side chain.

Authors:  I Björkhem; J Gustafsson; G Johansson; B Persson
Journal:  J Clin Invest       Date:  1975-03       Impact factor: 14.808

8.  The metabolism of cholestanol, cholesterol, and bile acids in cerebrotendinous xanthomatosis.

Authors:  G Salen; S M Grundy
Journal:  J Clin Invest       Date:  1973-11       Impact factor: 14.808

9.  A biochemical abnormality in cerebrotendinous xanthomatosis. Impairment of bile acid biosynthesis associated with incomplete degradation of the cholesterol side chain.

Authors:  T Setoguchi; G Salen; G S Tint; E H Mosbach
Journal:  J Clin Invest       Date:  1974-05       Impact factor: 14.808

  9 in total
  8 in total

1.  Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosis.

Authors:  G Salen; S Shefer; F W Cheng; B Dayal; A K Batta; G S Tint
Journal:  J Clin Invest       Date:  1979-01       Impact factor: 14.808

2.  Demonstration of 26-hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosis.

Authors:  S Skrede; I Björkhem; E A Kvittingen; M S Buchmann; S O Lie; C East; S Grundy
Journal:  J Clin Invest       Date:  1986-09       Impact factor: 14.808

3.  Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study.

Authors:  I Björkhem; O Fausa; G Hopen; H Oftebro; J I Pedersen; S Skrede
Journal:  J Clin Invest       Date:  1983-01       Impact factor: 14.808

4.  Elevated cholesterol precursors other than cholestanol can also be a hallmark for CTX.

Authors:  M G M de Sain-van der Velden; A Verrips; B H C M T Prinsen; M de Barse; R Berger; G Visser
Journal:  J Inherit Metab Dis       Date:  2008-10-24       Impact factor: 4.982

5.  A 25-hydroxylation pathway of cholic acid biosynthesis in man and rat.

Authors:  S Shefer; F W Cheng; B Dayal; S Hauser; G S Tint; G Salen; E H Mosbach
Journal:  J Clin Invest       Date:  1976-04       Impact factor: 14.808

6.  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.

Authors:  G Salen; S Shefer; G S Tint; G Nicolau; B Dayal; A K Batta
Journal:  J Clin Invest       Date:  1985-08       Impact factor: 14.808

7.  Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid.

Authors:  H Oftebro; I Björkhem; S Skrede; A Schreiner; J I Pederson
Journal:  J Clin Invest       Date:  1980-06       Impact factor: 14.808

8.  Cerebrotendinous xanthomatosis: a review of biochemical findings of the patient population in The Netherlands.

Authors:  B J Koopman; B G Wolthers; J C van der Molen; W van der Slik; R J Waterreus; A van Spreeken
Journal:  J Inherit Metab Dis       Date:  1988       Impact factor: 4.982

  8 in total

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