title: The eyes of mito-mouse: mouse models of mitochondrial disease.
abstract: The recent creation of several mouse models of mitochondrial diseases has provided new insights into the understanding of human mitochondrial disorders. Whether these animals have clinical or histologic ophthalmologic abnormalities is of great interest given the high frequency of such abnormalities in humans with mitochondrial disorders. In this article, we describe the currently available mouse models for mitochondrial diseases with special emphasis on their ocular phenotype. These mouse models demonstrate multiple and varied ophthalmologic manifestations.
authors.affiliation: Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia, USA. vbiouss@emory.edu
authors.forename: Valérie
authors.initials: V
authors.lastname: Biousse
authors.forename: Machelle T
authors.initials: MT
authors.lastname: Pardue
authors.forename: Douglas C
authors.initials: DC
authors.lastname: Wallace
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Newman
mesh_terms: D000818:Animals; D002678:Chimera; D002701:Chloramphenicol / Q000494:pharmacology; D004268:DNA-Binding Proteins; D004195:Disease Models, Animal; D004351:Drug Resistance / Q000235:genetics; D005128:Eye Diseases / Q000235:genetics*; D006609:High Mobility Group Proteins; D051379:Mice; D018345:Mice, Knockout; D028361:Mitochondrial Diseases / Q000235:genetics*; D024101:Mitochondrial Proteins; D009154:Mutation; D009687:Nuclear Proteins / Q000172:deficiency / Q000235:genetics; D011500:Protein Synthesis Inhibitors / Q000494:pharmacology; D012097:Repressor Proteins / Q000235:genetics; D013482:Superoxide Dismutase / Q000235:genetics; D014157:Transcription Factors / Q000172:deficiency / Q000235:genetics; D014764:Viral Proteins / Q000235:genetics; D000099156:Superoxide Dismutase 2
chemical_list: D002701:Chloramphenicol; D004268:DNA-Binding Proteins; D006609:High Mobility Group Proteins; D024101:Mitochondrial Proteins; D009687:Nuclear Proteins; D011500:Protein Synthesis Inhibitors; D012097:Repressor Proteins; D013482:Superoxide Dismutase; D014157:Transcription Factors; D014764:Viral Proteins; D000099156:Superoxide Dismutase 2; C081160:Ant protein, Enterobacteria phage P22; C081930:Tfam protein, mouse; C422562:mitochondrial transcription factor A