title: Animal models for respiratory chain disease.
abstract: Elucidation of the pathogenesis in respiratory chain diseases is of great importance for developing specific treatments. The limitations inherent to the use of patient material make studies of human tissues often difficult and the mouse has therefore emerged as a suitable model organism for studies of respiratory chain diseases. In this review, we present an overview of the field and discuss in depth a few examples of animal models reproducing pathology of human disease with primary and secondary respiratory chain involvement.
authors.affiliation: Department of Medical Nutrition, Karolinska Institutet, NOVUM, Huddinge Hospital, S-141 86 Huddinge, Sweden. Nils_Goran.Larsson@mednut.ki.se
authors.forename: N G
authors.initials: NG
authors.lastname: Larsson
authors.forename: P
authors.initials: P
authors.lastname: Rustin
mesh_terms: D000818:Animals; D019069:Cell Respiration / Q000235:genetics; D004268:DNA-Binding Proteins; D004195:Disease Models, Animal; D005621:Friedreich Ataxia / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D006609:High Mobility Group Proteins; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D028361:Mitochondrial Diseases / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology*; D024101:Mitochondrial Proteins; D009154:Mutation / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism; D014157:Transcription Factors / Q000235:genetics / Q000378:metabolism
chemical_list: D004268:DNA-Binding Proteins; D006609:High Mobility Group Proteins; D024101:Mitochondrial Proteins; D009687:Nuclear Proteins; C499631:TFAM protein, human; C081930:Tfam protein, mouse; D014157:Transcription Factors; C422562:mitochondrial transcription factor A