title: Effect of resveratrol on cultured skin fibroblasts from patients with oxidative phosphorylation defects.
abstract: Few therapeutic options are available to patients with oxidative phosphorylation disorders. Administering pharmacological agents that are able to stimulate mitochondrial biogenesis have been put forward as a possible treatment, yet the approach remains in need of thorough testing. We investigated the effect of resveratrol in an in vitro setting. Mitochondrial enzymatic activities were tested in cultured skin fibroblasts from patients harboring a nuclear defect in either complex II or complex IV (n = 11), and in fibroblasts from healthy controls (n = 11). In the latter, preincubation with resveratrol resulted in a significant increase of citrate synthase, complex II and complex IV enzyme activity. In patients with complex II or complex IV deficiency, however, activity of the deficient complex could not be substantially augmented, and response was dependent upon the residual activity. We conclude that resveratrol is not capable of normalizing oxidative phosphorylation activities in deficient cell lines.
authors.affiliation: Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Ghent University Hospital, Ghent, Belgium.
authors.forename: Boel
authors.initials: B
authors.lastname: De Paepe
authors.forename: Katrien
authors.initials: K
authors.lastname: Vandemeulebroecke
authors.forename: Joél
authors.initials: J
authors.lastname: Smet
authors.forename: Arnaud
authors.initials: A
authors.lastname: Vanlander
authors.forename: Sara
authors.initials: S
authors.lastname: Seneca
authors.forename: Willy
authors.initials: W
authors.lastname: Lissens
authors.forename: Johan Lk
authors.initials: JL
authors.lastname: Van Hove
authors.forename: Ellen
authors.initials: E
authors.lastname: Deschepper
authors.forename: Paz
authors.initials: P
authors.lastname: Briones
authors.forename: Rudy
authors.initials: R
authors.lastname: Van Coster
mesh_terms: D002478:Cells, Cultured; D002950:Citrate (si)-Synthase / Q000378:metabolism; D030401:Cytochrome-c Oxidase Deficiency / Q000201:enzymology* / Q000503:physiopathology; D042963:Electron Transport Complex II / Q000172:deficiency* / Q000378:metabolism; D003576:Electron Transport Complex IV / Q000235:genetics / Q000378:metabolism; D005347:Fibroblasts / Q000187:drug effects* / Q000201:enzymology; D006801:Humans; D008928:Mitochondria / Q000187:drug effects / Q000201:enzymology; D010085:Oxidative Phosphorylation / Q000187:drug effects*; D000077185:Resveratrol; D013267:Stilbenes / Q000494:pharmacology*
chemical_list: D013267:Stilbenes; D042963:Electron Transport Complex II; D003576:Electron Transport Complex IV; D002950:Citrate (si)-Synthase; D000077185:Resveratrol
keywords: mitochondrial biogenesis; oxidative phosphorylation; resveratrol