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Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency.
Peroxisomes play an essential role in a number of different metabolic pathways, including the beta-oxidation of a distinct set of fatty acids and fatty acid derivatives. The importance of theExpand
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Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
In the past few years, many patients have been described who have a defect of unknown origin in the peroxisomal beta-oxidation pathway. Complementation analysis has been done by various groups toExpand
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Bifunctional protein deficiency: Complementation within the same group suggesting differential enzyme defects and clues to the underlying basis
E. G. VAN GRUNSVEN1, E. VAN BERKEL1, H. LEMONDE2, P. T. CLAYTON2 and R. J. A. WANDERS1,3* of Medical of 1 Clinical University Amsterdam, Academic Center, Departments Chemistry and ChildrenÏsExpand
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The Role of NF-κB in TNF-Related Apoptosis-Inducing Ligand (TRAIL)-Induced Apoptosis of Melanoma Cells1
Previous studies have shown that activation of NF-κB can inhibit apoptosis induced by a number of stimuli. It is also known that TNF-related apoptosis-inducing ligand (TRAIL) can activate NF-κBExpand
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Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx). Activity measurements in liver and fibroblasts using a newly developed method.
Sterol carrier protein X (SCPx) plays a crucial role in the peroxisomal oxidation of branched-chain fatty acids. To investigate whether patients with an unresolved defect in peroxisomalExpand
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Identification of the newly discovered 58kDa peroxisomal thiolase SCPx as the main thiolase involved in both pristanic acid and trihydroxycholestanoic acid oxidation: Implications for peroxisomal
R. J. A. WANDERS1,2*, S. DENIS1, E. VAN BERKEL1, F. WOUTERS3, K. W. A. WIRTZ3 and U. SEEDORF4 of Amsterdam, Academic Medical Centre, Departments of 1 Clinical University Chemistry and 2 Pediatrics,Expand
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Homeobox proteins as signal transduction intermediates in regulation of NCAM expression by recombinant human bone morphogenetic protein-2 in osteoblast-like cells.
The role of homeobox genes in signaling of recombinant human bone morphogenetic protein-2 (rhBMP-2) was studied in osteoblast-like cells. Expression of several homeobox genes was decreased byExpand
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D-hydroxyacyl-CoA dehydrogenase deficiency. Identification of a new peroxisomal disorder with implications for other disorders of beta-oxidation.
The second and third steps of peroxisomal beta-oxidation are catalysed by two multifunctional enzymes: D-bifunctional protein and L-bifunctional protein. Here we show that fibroblasts of a patientExpand