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- Publications
- Influence
Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency.
- E. G. van Grunsven, E. van Berkel, +7 authors R. Wanders
- Biology, Medicine
- Proceedings of the National Academy of Sciences…
- 3 March 1998
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 the… Expand
Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis.
- E. G. van Grunsven, E. van Berkel, +8 authors R. Wanders
- Biology, Medicine
- American journal of human genetics
- 1999
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 to… Expand
Bifunctional protein deficiency: Complementation within the same group suggesting differential enzyme defects and clues to the underlying basis
- E. G. van Grunsven, E. van Berkel, H. Lemonde, P. Clayton, R. Wanders
- Biology, Medicine
- Journal of Inherited Metabolic Disease
- 1 June 1998
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 ChildrenÏs… Expand
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-κB… Expand
Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx). Activity measurements in liver and fibroblasts using a newly developed method.
- S. Ferdinandusse, S. Denis, E. van Berkel, G. Dacremont, R. Wanders
- Biology, Medicine
- Journal of lipid research
- 1 March 2000
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 peroxisomal… Expand
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. Wanders, S. Denis, E. van Berkel, F. Wouters, K. Wirtz, U. Seedorf
- Chemistry, Medicine
- Journal of Inherited Metabolic Disease
- 1 June 1998
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
Homeobox proteins as signal transduction intermediates in regulation of NCAM expression by recombinant human bone morphogenetic protein-2 in osteoblast-like cells.
- C. J. Boersma, M. Bloemen, J. M. Hendriks, E. van Berkel, W. Olijve, E. J. Van zoelen
- Biology, Medicine
- Molecular cell biology research communications…
- 1 May 1999
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 by… Expand
D-hydroxyacyl-CoA dehydrogenase deficiency. Identification of a new peroxisomal disorder with implications for other disorders of beta-oxidation.
- E. G. van Grunsven, E. van Berkel, S. Denis, P. Mooijer, R. Wanders
- Biology, Medicine
- Advances in experimental medicine and biology
- 1999
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 patient… Expand