Maria de Fátima Vieira Ventura
PhD (1998) in Pharmacy (Biochemistry), Universidade de Lisboa
Assistant Professor, Biochemistry and Human Biology
Faculdade de Farmácia, Universidade de Lisboa — Av. Prof. Gama Pinto, 1649-003 Lisboa, Portugal
Elucidation of the pathogenesis of mitochondrial fatty acid beta-oxidation (FAO) disorders, namely the interaction mechanisms between FAO, energy metabolism and the carnitine cycle. In vitro and in silico characterization of the wild-type and various disease-causing mutants of the medium-chain acyl-CoA dehydrogenase is ongoing. The ultimate goal is to define structural-functional markers for the screening for small molecules able to rescue the mutants showing vitro to be affected by misfolding.
Houten SM, Violante S, Ventura FV, Wanders RJ. The biochemistry and physiology of mitochondrial fatty acid ß-oxidation and its genetic disorders. Annu Rev Physiol 2016; 78:23-44.
Violante S, Ijlst L, Te Brinke H, Koster J, Tavares de Almeida I, Wanders RJ, Ventura FV, Houten SM. Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient. Biochim Biophys Acta 2013; 1831: 1467-74.
Violante S, Ijlst L, Te Brinke H, Tavares de Almeida I, Wanders RJ, Ventura FV, Houten SM. Carnitine palmitoyltransferase 2 and carnitine/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines. FASEB J 2013; 27:2039-44.
Bonito CA, Leandro P, Ventura FV, Guedes RC. Insights into Medium-chain Acyl-CoA Dehydrogenase Structure by Molecular Dynamics Simulations. Chem Biol Drug Des 2016; 88:281-92.
Bonito CA, Nunes J, Leandro J, Louro F, Leandro P, Ventura FV, Guedes RC. Unveiling the Pathogenic Molecular Mechanisms of the Most Common Variant (p.K329E) in Medium-Chain Acyl-CoA Dehydrogenase Deficiency by in Vitro and in Silico Approaches. Biochemistry 2016; 55: 7086-98.