title: Mouse models for mitochondrial diseases.
abstract: Mitochondrial diseases are heterogeneous and incurable conditions typically resulting from deficient ATP production in the cells. Mice, owing to their genetic and physiological similarity to humans as well as their relatively easy maintenance and propagation, are extremely valuable for studying mitochondrial diseases and are also indispensable for the preclinical evaluation of novel therapies for these devastating conditions. Here, we review the recent exciting developments in the field focusing on mouse models for mitochondrial disease genes although models for genes not involved in the pathogenesis of mitochondrial disease and therapeutic proof-of-concept studies using mouse models are also discussed.
authors.affiliation: INSERM U1163, Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine, 75015 Paris, France.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Ruzzenente
authors.affiliation: INSERM U1163, Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine, 75015 Paris, France.
authors.forename: Agnès
authors.initials: A
authors.lastname: Rötig
authors.affiliation: INSERM U1163, Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine, 75015 Paris, France metodi.metodiev@inserm.fr.
authors.forename: Metodi D
authors.initials: MD
authors.lastname: Metodiev