Literature DB >> 2122102

Di- and trihydroxycholestanoic acidaemia with hepatic failure.

H Przyrembel1, R J Wanders, C W van Roermund, R B Schutgens, G P Mannaerts, M Casteels.   

Abstract

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Year:  1990        PMID： 2122102     DOI： 10.1007/bf01799397

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  11 in total

Review 1.  The inborn errors of peroxisomal beta-oxidation: a review.

Authors:  R J Wanders; C W van Roermund; R B Schutgens; P G Barth; H S Heymans; H van den Bosch; J M Tager
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

2.  Inhibition of 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid oxidation and of bile acid secretion in rat liver by fatty acids.

Authors:  M Casteels; L Schepers; J Van Eldere; H J Eyssen; G P Mannaerts
Journal:  J Biol Chem       Date:  1988-04-05       Impact factor: 5.157

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

Authors:  K D Setchell; F J Suchy; M B Welsh; L Zimmer-Nechemias; J Heubi; W F Balistreri
Journal:  J Clin Invest       Date:  1988-12       Impact factor: 14.808

4.  The metabolism of 3alpha, 7alpha, 12alpha-trihydorxy-5beta-cholestan-26-oic acid in two siblings with cholestasis due to intrahepatic bile duct anomalies. An apparent inborn error of cholic acid synthesis.

Authors:  R F Hanson; J N Isenberg; G C Williams; D Hachey; P Szczepanik; P D Klein; H L Sharp
Journal:  J Clin Invest       Date:  1975-09       Impact factor: 14.808

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

Authors:  P T Clayton; J V Leonard; A M Lawson; K D Setchell; S Andersson; B Egestad; J Sjövall
Journal:  J Clin Invest       Date:  1987-04       Impact factor: 14.808

6.  Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency.

Authors:  A W Schram; S Goldfischer; C W van Roermund; E M Brouwer-Kelder; J Collins; T Hashimoto; H S Heymans; H van den Bosch; R B Schutgens; J M Tager
Journal:  Proc Natl Acad Sci U S A       Date:  1987-04       Impact factor: 11.205

7.  Peroxisomal bifunctional enzyme deficiency.

Authors:  P A Watkins; W W Chen; C J Harris; G Hoefler; S Hoefler; D C Blake; A Balfe; R I Kelley; A B Moser; M E Beard
Journal:  J Clin Invest       Date:  1989-03       Impact factor: 14.808

8.  A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy).

Authors:  B T Poll-The; F Roels; H Ogier; J Scotto; J Vamecq; R B Schutgens; R J Wanders; C W van Roermund; M J van Wijland; A W Schram
Journal:  Am J Hum Genet       Date:  1988-03       Impact factor: 11.025

9.  Subcellular distribution and characteristics of trihydroxycoprostanoyl-CoA synthetase in rat liver.

Authors:  L Schepers; M Casteels; K Verheyden; G Parmentier; S Asselberghs; H J Eyssen; G P Mannaerts
Journal:  Biochem J       Date:  1989-01-01       Impact factor: 3.857

10.  Peroxisomal beta-oxidation of palmitoyl-CoA in human liver homogenates and its deficiency in the cerebro-hepato-renal (Zellweger) syndrome.

Authors:  R J Wanders; C W van Roermund; C T de Vries; H van den Bosch; G Schrakamp; J M Tager; A W Schram; R B Schutgens
Journal:  Clin Chim Acta       Date:  1986-08-30       Impact factor: 3.786
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  5 in total

1.  Di- and trihydroxycholestanaemia in twin sisters.

Authors:  R J Wanders; C W van Roermund; A Schelen; R B Schutgens; J Zeman; V Kozich; J Hyanek; M Casteels; G P Mannaerts
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

Review 2.  Biochemistry of peroxisomes in health and disease.

Authors:  I Singh
Journal:  Mol Cell Biochem       Date:  1997-02       Impact factor: 3.396

3.  Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger syndrome.

Authors:  E Baumgart; J C Vanhooren; M Fransen; P Marynen; M Puype; J Vandekerckhove; J A Leunissen; H D Fahimi; G P Mannaerts; P P van Veldhoven
Journal:  Proc Natl Acad Sci U S A       Date:  1996-11-26       Impact factor: 11.205

Review 4.  Peroxisomal disorders: a review.

Authors:  B Fournier; J A Smeitink; L Dorland; R Berger; J M Saudubray; B T Poll-The
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982

Review 5.  Inborn errors of bile acid metabolism.

Authors:  P T Clayton
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982
  5 in total

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