Journal
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
Volume 459, Issue 1, Pages 66-70Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.bbrc.2015.02.070
Keywords
G7731A mtDNA mutation; Mitochondrial tRNA(Lys) gene; Transmitochondrial mice; Respiration defects; Late-onset disorders; Mitochondrial disease models
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Funding
- Japan Society for the Promotion of Science [25250011, 23240058, 24117503]
- World Premier International Research Center Initiative, Ministry of Education, Culture, Sports, Science, and Technology Japan
- Grants-in-Aid for Scientific Research [25250011, 24117503, 23240058] Funding Source: KAKEN
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We previously generated mito-mice-tRNA(Lys7731) as a model for primary prevention of mitochondrial diseases. These mice harbour a G7731A mtDNA mutation in the tRNA(Lys) gene, but express only muscle weakness and short body length by four months. Here, we examined the effects of their aging on metabolic and histologic features. Unlike young mito-mice-tRNA(Lys7731), aged mito-mice-tRNA(Lys7731) developed muscle atrophy, renal failures, and various metabolic abnormalities, such as lactic acidosis and anemia, characteristic of patients with mitochondrial diseases. These observations provide convincing evidence that the respiration defects induced by high G7731A mtDNA levels cause these late-onset disorders that are relevant to mitochondrial diseases. (C) 2015 Elsevier Inc. All rights reserved.
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