title: Mitochondrial DNA disease-molecular insights and potential routes to a cure.
abstract: Mitochondrial DNA diseases are common neurological conditions caused by mutations in the mitochondrial genome or nuclear genes responsible for its maintenance. Current treatments for these disorders are focussed on the management of the symptoms, rather than the correction of biochemical defects caused by the mutation. This review focuses on the molecular effects of mutations, the symptoms they cause and current work focusing on the development of targeted treatments for mitochondrial DNA disease.
authors.affiliation: Newcastle University Centre for Brain Ageing and Vitality and Wellcome Trust Centre for Mitochondrial Research, Institute for Ageing and Health, The Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.
authors.forename: Oliver
authors.initials: O
authors.lastname: Russell
authors.affiliation: Newcastle University Centre for Brain Ageing and Vitality and Wellcome Trust Centre for Mitochondrial Research, Institute for Ageing and Health, The Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne NE2 4HH, UK. Electronic address: doug.turnbull@newcastle.ac.uk.
authors.forename: Doug
authors.initials: D
authors.lastname: Turnbull
mesh_terms: D004272:DNA, Mitochondrial / Q000235:genetics; D006801:Humans; D008928:Mitochondria / Q000201:enzymology / Q000235:genetics; D028361:Mitochondrial Diseases / Q000235:genetics* / Q000628:therapy; D025261:Mitochondrial Proton-Translocating ATPases / Q000235:genetics; D017354:Point Mutation; D012356:RNA, Transfer, Leu / Q000235:genetics; D017384:Sequence Deletion
chemical_list: D004272:DNA, Mitochondrial; D012356:RNA, Transfer, Leu; C412204:ATP synthase subunit 6; D025261:Mitochondrial Proton-Translocating ATPases
keywords: Mitochondrial disease; TALENs; Treatments; Zinc-finger nucleases