Literature DB >> 9686380

Bifunctional protein deficiency: complementation within the same group suggesting differential enzyme defects and clues to the underlying basis.

E G Van Grunsven1, E van Berkel, H Lemonde, P T Clayton, R J Wanders.   

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Year:  1998        PMID： 9686380     DOI： 10.1023/a:1005396912015

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


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  10 in total

1.  Physiological role of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein.

Authors:  L L Jiang; T Kurosawa; M Sato; Y Suzuki; T Hashimoto
Journal:  J Biochem       Date:  1997-03       Impact factor: 3.387

2.  Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme protein.

Authors:  E G van Grunsven; C W van Roermund; S Denis; R J Wanders
Journal:  Biochem Biophys Res Commun       Date:  1997-06-09       Impact factor: 3.575

3.  Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects.

Authors:  E G van Grunsven; R J Wanders
Journal:  J Inherit Metab Dis       Date:  1997-07       Impact factor: 4.982

4.  Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry.

Authors:  N M Verhoeven; W Kulik; C M van den Heuvel; C Jakobs
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

5.  Sterol carrier protein X (SCPx) is a peroxisomal branched-chain beta-ketothiolase specifically reacting with 3-oxo-pristanoyl-CoA: a new, unique role for SCPx in branched-chain fatty acid metabolism in peroxisomes.

Authors:  R J Wanders; S Denis; F Wouters; K W Wirtz; U Seedorf
Journal:  Biochem Biophys Res Commun       Date:  1997-07-30       Impact factor: 3.575

Review 6.  Peroxisomal disorders: a review.

Authors:  R J Wanders; R B Schutgens; P G Barth
Journal:  J Neuropathol Exp Neurol       Date:  1995-09       Impact factor: 3.685

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.  Plasma bile acids in patients with peroxisomal dysfunction syndromes: analysis by capillary gas chromatography-mass spectrometry.

Authors:  P T Clayton; B D Lake; N A Hall; D B Shortland; R A Carruthers; A M Lawson
Journal:  Eur J Pediatr       Date:  1987-03       Impact factor: 3.183

9.  Properties of mitochondria and peroxisomal enoyl-CoA hydratases from rat liver.

Authors:  S Furuta; S Miyazawa; T Osumi; T Hashimoto; N Ui
Journal:  J Biochem       Date:  1980-10       Impact factor: 3.387

10.  The reactions catalyzed by the inducible bifunctional enzyme of rat liver peroxisomes cannot lead to the formation of bile acids.

Authors:  R Xu; D A Cuebas
Journal:  Biochem Biophys Res Commun       Date:  1996-04-16       Impact factor: 3.575
  10 in total

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