Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid (Q28367184)

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English	Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid	scientific article

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instance of

scholarly article

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title

Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid (English)

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main subject

mitochondrion

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based on heuristic

inferred from title

cerebrotendinous xanthomatosis

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based on heuristic

inferred from title

author

Ingemar Björkhem

series ordinal

2

object named as

I Björkhem

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author name string

H Oftebro

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1

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S Skrede

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3

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A Schreiner

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4

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J I Pederson

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5

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language of work or name

English

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publication date

June 1980

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published in

Journal of Clinical Investigation

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volume

65

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issue

6

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page(s)

1418-30

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cites work

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

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

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29 September 2017

Formation of bile acids in man: conversion of cholesterol into 5-beta-cholestane-3-alpha, 7-alpha, 12-alpha-triol in liver homogenates

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

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29 September 2017

Bile Acid Metabolism

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Biosynthesis and metabolism of bile acids in man

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29 September 2017

Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosis

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Biosynthesis of chenodeoxycholic acid in man: stereospecific side-chain hydroxylations of 5beta-cholestane-3alpha,7alpha-diol

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On the 25-hydroxylation of vitamin D3 in vitro studied with a mass fragmentographic technique

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Reconstitution of vitamin D3 25-hydroxylase activity with a cytochrome P-450 preparation from rat liver mitochondria

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29 September 2017

Quantitative aspects of the conversion of 5 beta-cholestane intermediates to bile acids in man

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29 September 2017

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

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Assay and properties of a mitochondrial 25-hydroxylase active on vitamine D3

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Partial synthesis of the two 3alpha:7alpha:12alpha-trihydroxycoprostanic acids and of similar bile acids with extended chains

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Comparative studies of bile salts. V. Bile salts of Crocodylidae

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2 June 2018

Cholestanol deposition in cerebrotendinous xanthomatosis. A possible mechanism

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2 June 2018

Cerebrotendinous xanthomatosis (cholestanolosis). Investigations on two sisters and their family

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2 June 2018

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

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2 June 2018

26-Hydroxylation of C27-steroids by soluble liver mitochondrial cytochrome P-450

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Structural and Metabolic Heterogeneity of Rat Liver Glycerophosphatides

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SYNTHESIS AND METABOLISM OF CHOLEST-4-ENE-7-ALPHA,12-ALPHA-DIOL-3-ONE AND 5-BETA-CHOLESTANE-7-ALPHA,12-ALPHA-DIOL-3-ONE. BILE ACIDS AND STEROIDS 153

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Carbon-13 NMR studies on cholesterol biosynthesized from [13C]mevalonates

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On the stereospecificity of microsomal “26”-hydroxylation in bile acid biosynthesis

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ON THE STEREOSPECIFICITY OF 26-HYDROXYLATION OF CHOLESTEROL. BILE ACIDS AND STEROIDS. 155

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reference URL

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12 December 2020

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Crystallization of trihydroxycoprostani acid from human bile

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12 December 2020

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Omega-hydroxylation of steriod side-chain in biosynthesis of bile acids

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12 December 2020

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Purification of NADPH-ferredoxin reductase from rat liver mitochondria

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https://pubmed.ncbi.nlm.nih.gov/7410549

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12 December 2020

based on heuristic

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Formation of trihydroxycoprostanic acid from cholesterol in man

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reference URL

https://pubmed.ncbi.nlm.nih.gov/7410549

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12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Enzymatic characteristics of CO-sensitive 26-hydroxylase system for 5beta-cholestane-3 alpha, 7 alpha, 12 alpha-triol in rat-liver mitochondria and its intramitochondrial localization

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PubMed

reference URL

https://pubmed.ncbi.nlm.nih.gov/7410549

retrieved

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

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PubMed

reference URL

https://pubmed.ncbi.nlm.nih.gov/7410549

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12 December 2020

based on heuristic

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A novel specific assay of 25-hydroxy vitamin D

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PubMed

reference URL

https://pubmed.ncbi.nlm.nih.gov/7410549

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12 December 2020

based on heuristic

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Identifiers

DOI

10.1172/JCI109806

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PMC publication ID

371480

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PubMed publication ID

7410549

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