9 documents found, page 1 of 1
Substrate specificity of Carnitine Palmitoyltransferase 2
Violante, S; IJlst, L; Tavares de Almeida, I; Wanders, RJ; Ventura, FV
Role of Long-chain Fatty Acyl-Coenzyme A Esters in the Pathogenic Mechanisms of...
Ventura, FV; Violante, S; Ruiter, J; IJlst, L; Silva, MJ; Houten, SM; Tavares de Almeida, I; Wanders, RJ
Pathogenesis of Mitocondrial Long-Chain Fatty Acids ß-Oxidation Disorders
Violante, S; Ruiter, J; IJlst, L; Silva, MJ; Houten, SM; Tavares de Almeida, I; Wanders, RJ; Ventura, FV
Carnitine Palmitoyltransferase 2 Revisited
Violante, S; IJlst, L; Tavares de Almeida, I; Wanders, RJ; Ventura, FVFCT. - SFRH/BD/38074/2007
Heterologous expression of the human carnitine acylcarnitine translocase (CACT)
Ventura, FV; Violante, S; Jorge, AM; IJlst, L; Gaspar, MM; van Roermund, CWT; Cruz, MEM; Soveral, G; Wanders, RJA; Leandro, P; Tavares de Almeida, I
Mitochondrial long-chain acyl-CoA ß-oxidation intermediates
Ventura, FV; Ruiter, J; IJlst, L; Tavares de Almeida, I; Wanders, RJA
Pathogenic mechanisms of long-chain fatty acid ß-oxidation defects
Ventura, FV; Ruiter, J; IJlst, L; Tavares de Almeida, I; Wanders, RJA
Differential effect of valproate and its Delta(2)- and Delta(4)-unsaturated met...
Silva, MFB; Ruiter, JPN; IJlst, L; Jakobs, C; Duran, M; de Almeida, IT; Wanders, RJAOverall fatty acid oxidation rates were investigated in rat hepatocytes using [9,10-H-3]palmitic, [9,10-H-3]-oleic, [9.10-H-3]-myristic and [2,3-H-3]-phenylpropionic acids. The effect of both valproate (VPA) (0-10 mM) and two of its unsaturated metabolite
Synthesis and intramitochondrial levels of valproyl-coenzyme A metabolites
Silva, MFB; Ruiter, JPN; IJlst, L; Allers, P; ten Brink, HJ; Jakobs, C; Duran, M; de Almedia, IT; Wander, RJAA number of valproate adverse reactions are due to its interference with several metabolic pathways, including that of fatty acid oxidation. In order to resolve which mitochondrial enzymes of fatty acid oxidation are inhibited by which VPA intermediates w