Delta 4-3-oxosteroid 5 beta-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis (Q28361181)

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English	Delta 4-3-oxosteroid 5 beta-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis	scientific article

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scholarly article

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Delta 4-3-oxosteroid 5 beta-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis (English)

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author name string

K D Setchell

1

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F J Suchy

2

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M B Welsh

3

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L Zimmer-Nechemias

4

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J Heubi

5

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W F Balistreri

6

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language of work or name

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publication date

December 1988

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Journal of Clinical Investigation

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82

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6

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2148-57

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The bile acid composition of gastric contents from neonates with high intestinal obstruction

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20 March 2017

Bile Acid Synthesis in Man: Metabolism of 7α-Hydroxycholesterol-14C and 26-Hydroxycholesterol-3H

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20 March 2017

Familial giant cell hepatitis associated with synthesis of 3 beta, 7 alpha-dihydroxy-and 3 beta,7 alpha, 12 alpha-trihydroxy-5-cholenoic acids

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20 March 2017

Purification and properties of a 3 beta-hydroxy-delta 5-C27-steroid oxidoreductase from rabbit liver microsomes

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29 September 2017

Diversity of bile acids in the fetus and newborn infant

1 reference

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29 September 2017

Serum bile acid analysis

1 reference

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29 September 2017

Effect of Biliary Drainage on Individual Reactions in the Conversion of Cholesterol to Taurocholic Acid. Bile Acids and Steroids 180

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29 September 2017

Effect of sodium taurolithocholate on bile flow and bile acid exeretion

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29 September 2017

Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis

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29 September 2017

Bile acid profiles in urine of patients with liver diseases

1 reference

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29 September 2017

Tetrahydroxylated bile acids in healthy human newborns

1 reference

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2 June 2018

Bile-salt metabolism in the newborn. Measurement of pool size and synthesis by stable isotope technic

1 reference

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2 June 2018

Bile acid synthesis in rat liver peroxisomes: metabolism of 26-hydroxycholesterol to 3 beta-hydroxy-5-cholenoic acid.

1 reference

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2 June 2018

Bile acid therapies applied to patients suffering from cerebrotendinous xanthomatosis.

1 reference

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2 June 2018

Fast atom bombardment mass spectrometry in the diagnosis of cerebrotendinous xanthomatosis.

1 reference

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2 June 2018

Bile acid metabolism during development: metabolism of lithocholic acid in human fetal liver

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=442799

2 June 2018

Hepatic bile acid metabolism during early development revealed from the analysis of human fetal gallbladder bile

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=442799

2 June 2018

Rapid diagnosis of Zellweger syndrome and infantile Refsum's disease by fast atom bombardment--mass spectrometry of urine bile salts.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=442799

2 June 2018

Developmental pattern of bile acid metabolism as revealed by bile acid analysis of meconium.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=442799

27 November 2018

On the conversion of cholesterol to 7-alpha,12-alpha-dihydroxycholest-4-en-3-one. Bile acids and steroids 168

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

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BILE ACIDS. XXII. ALLOCHOLIC ACID, A METABOLITE OF 5-ALPHA-CHOLESTAN-3-BET-A-OL IN THE RAT

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

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Conversion of cholesterol to bile acids in rat: purification and properties of a delta-4-3-ketosteroid 5-beta-reductase and a 3-alpha-hydroxysteroid dehydrogenase

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

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Enzymatic conversion of a delta-4-3-ketosteroid into a 3-alpha-hydroxy-5-beta steroid: mechanism and stereochemistry of hydrogen transfer from NADPH. Bile acids and steroids 190

1 reference

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12 December 2020

based on heuristic

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Metabolism of cholest-5-ene-3 beta, 26-diol in the rat and hamster

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

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Formation and metabolism of 7 alpha-hydroxy-5-alpha-cholestan-3-one and 7 alpha, 12 alpha-dihydroxy-5-alpha-cholestan-3-one in rat liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

12alpha-hydroxylation of 7alpha-hydroxy-4-cholesten-3-one by a reconstituted system from rat liver microsomes

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

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Bile acid profiles of human serum and skin interstitial fluid and their relationship to pruritus studied by gas chromatography-mass spectrometry

1 reference

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based on heuristic

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A rapid method for the quantitative extraction of bile acids and their conjugates from serum using commercially available reverse-phase octadecylsilane bonded silica cartridges

1 reference

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12 December 2020

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inferred from PubMed ID database lookup

Inborn errors of steroid biosynthesis: detection by a new mass-spectrometric method

1 reference

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based on heuristic

inferred from PubMed ID database lookup

Analysis of intact steroid conjugates by secondary ion mass spectrometry (including FABMS) and by gas chromatography

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Bile acid metabolism during development: metabolism of taurodeoxycholic acid in human fetal liver

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Studies on the conjugating activity of bile acids in children

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Biliary and urinary excretion of sulfated, glucuronidated and tetrahydroxylated bile acids in cirrhotic patients

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Capillary gas chromatography of hydroxylated bile acid stereoisomers of the allo and normal series

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Inborn errors of bile acid synthesis.

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Analysis of 3-sulfated and nonsulfated bile acids by one-step solvolysis and high performance liquid chromatography

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

On the bile acids in duodenal contents of infants and children. Bile acids and steroids 72

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

3-Oxo-delta 4 bile acids in liver disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

IDENTIFICATION OF ALLODEOXYCHOLIC ACID AS THE MAJOR COMPONENT OF GALLSTONES INDUCED IN THE RABBIT BY 5-ALPHA-CHOLESTAN-3-BETA-OL

1 reference

https://pubmed.ncbi.nlm.nih.gov/3198770

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1172/JCI113837

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