title: Yeast models of human mitochondrial diseases: from molecular mechanisms to drug screening.
abstract: Mitochondrial diseases are rare diseases most often linked to energy in the form of ATP-depletion. The high number of nuclear- and mitochondrial-DNA-encoded proteins (>500), required for ATP production and other crucial mitochondrial functions such as NADH re-oxidation, explains the increasing number of reported disorders. In recent years, yeast has revealed to be a powerful model to identify responsible genes, to study primary effects of pathogenic mutations and to determine the molecular mechanisms leading to mitochondrial disorders. However, the clinical management of patients with mitochondrial disorders is still essentially supportive. Here we review some of the most fruitful yeast mitochondrial disorder models and propose to subject these models to highthroughput chemical library screening to prospect new therapeutic drugs against mitochondrial diseases.
authors.affiliation: IBGC-CNRS/Université Bordeaux 2, Bordeaux, France.
authors.forename: Christine
authors.initials: C
authors.lastname: Schwimmer
authors.forename: Malgorzata
authors.initials: M
authors.lastname: Rak
authors.forename: Linnka
authors.initials: L
authors.lastname: Lefebvre-Legendre
authors.forename: Stéphane
authors.initials: S
authors.lastname: Duvezin-Caubet
authors.forename: Guillaume
authors.initials: G
authors.lastname: Plane
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: di Rago
mesh_terms: D000818:Animals; D001681:Biological Assay / Q000379:methods; D016503:Drug Delivery Systems / Q000379:methods; D015195:Drug Design; D004353:Drug Evaluation, Preclinical / Q000379:methods*; D006801:Humans; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism*; D028361:Mitochondrial Diseases / Q000188:drug therapy* / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000378:metabolism*; D008967:Molecular Biology / Q000379:methods; D012441:Saccharomyces cerevisiae / Q000187:drug effects* / Q000378:metabolism*
chemical_list: D024101:Mitochondrial Proteins