muscle
• mice show some disruption of normal muscle architecture with presence of abnormal myofibril bundles and centralized nuclei, indicative of persistent muscle damage
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• no mice successfully complete the hanging wire test, with 50% of mice falling just after 60 seconds and the longest hanging time is just over 100 seconds compared to wild-type mice that can hang for 3 minutes and mice are inactive for 15-30 min after falling from the hanging wire
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behavior/neurological
• no mice successfully complete the hanging wire test, with 50% of mice falling just after 60 seconds and the longest hanging time is just over 100 seconds compared to wild-type mice that can hang for 3 minutes indicating impaired exercise tolerance
• mice are inactive for 15-30 min after falling from the hanging wire unlike wild-type mice which return to normal activity immediately after the test
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homeostasis/metabolism
• no mice successfully complete the hanging wire test, with 50% of mice falling just after 60 seconds and the longest hanging time is just over 100 seconds compared to wild-type mice that can hang for 3 minutes indicating impaired exercise tolerance
• mice are inactive for 15-30 min after falling from the hanging wire unlike wild-type mice which return to normal activity immediately after the test
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• mice exhibit higher resting L-lactic acid levels, more than double that of wild-type levels, and have over double the wild-type levels at the time of fall in the hanging wire test
• however, glucose levels are normal before and after the hanging wire test
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
nuclear type mitochondrial complex I deficiency 20 | DOID:0112072 |
OMIM:611126 |
J:326969 |