Literature DB >> 9266375

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

E G van Grunsven1, R J Wanders.   

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Year:  1997        PMID： 9266375     DOI： 10.1023/a:1005323221660

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


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  13 in total

1.  Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.

Authors:  R J Wanders; S Denis; J P Ruiter; R B Schutgens; C W van Roermund; B S Jacobs
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

Review 2.  Postnatal diagnosis of peroxisomal disorders: a biochemical approach.

Authors:  R J Wanders; R B Schutgens; P G Barth; J M Tager; H van den Bosch
Journal:  Biochimie       Date:  1993       Impact factor: 4.079

3.  Purification and properties of acyl-CoA oxidase from rat liver.

Authors:  T Osumi; T Hashimoto; N Ui
Journal:  J Biochem       Date:  1980-06       Impact factor: 3.387

4.  Peroxisomal beta-oxidation. Purification of four novel 3-hydroxyacyl-CoA dehydrogenases from rat liver peroxisomes.

Authors:  D K Novikov; G F Vanhove; H Carchon; S Asselberghs; H J Eyssen; P P Van Veldhoven; G P Mannaerts
Journal:  J Biol Chem       Date:  1994-10-28       Impact factor: 5.157

5.  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

6.  The CoA esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-CoA oxidase in human liver and kidney.

Authors:  G F Vanhove; P P Van Veldhoven; M Fransen; S Denis; H J Eyssen; R J Wanders; G P Mannaerts
Journal:  J Biol Chem       Date:  1993-05-15       Impact factor: 5.157

7.  Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters.

Authors:  J Mosser; A M Douar; C O Sarde; P Kioschis; R Feil; H Moser; A M Poustka; J L Mandel; P Aubourg
Journal:  Nature       Date:  1993-02-25       Impact factor: 49.962

8.  Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysis.

Authors:  S Brul; A Westerveld; A Strijland; R J Wanders; A W Schram; H S Heymans; R B Schutgens; H van den Bosch; J M Tager
Journal:  J Clin Invest       Date:  1988-06       Impact factor: 14.808

9.  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

10.  Sterol carrier protein X is peroxisomal 3-oxoacyl coenzyme A thiolase with intrinsic sterol carrier and lipid transfer activity.

Authors:  U Seedorf; P Brysch; T Engel; K Schrage; G Assmann
Journal:  J Biol Chem       Date:  1994-08-19       Impact factor: 5.157
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  2 in total

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

Authors:  E G Van Grunsven; E van Berkel; H Lemonde; P T Clayton; R J Wanders
Journal:  J Inherit Metab Dis       Date:  1998-06       Impact factor: 4.982

2.  Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.

Authors:  E G van Grunsven; E van Berkel; P A Mooijer; P A Watkins; H W Moser; Y Suzuki; L L Jiang; T Hashimoto; G Hoefler; J Adamski; R J Wanders
Journal:  Am J Hum Genet       Date:  1999-01       Impact factor: 11.025
  2 in total

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