mortality/aging
• deaths occur by the second week and no mice survive beyond 30 days
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growth/size/body
• normal size at birth
• growth retardation apparent by day 7
• organ weights of heart, liver, spleen, and kidney are normal through day 12
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homeostasis/metabolism
• exhibit shivering
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• colder than normal littermates
• become progressively and severely hypothermic from 10 days of age
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cellular
• 40-80% reduction in mitochondrial DNA levels in all organs investigated
• skeletal muscle, heart, liver, and spleen with normal mitochondrial DNA levels at birth but progressive loss during the first two weeks of life
• mitochondrial DNA levels already low at birth in the brain and adipose tissue
• mutation rate for mitochondrial DNA is normal
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• heart mitochondria show disrupted cristae at 14 days
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cardiovascular system
• muscle fibers in the heart are disorganized
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liver/biliary system
• increased numbers of intracellular small lipid vesicles at 14 days of age
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adipose tissue
• near complete absence of hypodermal fat layer at 14 days
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• altered structure of interscapular brown fat
• heterogeneity of cell size and accumulation of lipid vesicles at 14 days
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muscle
• muscle fibers in the heart are disorganized
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integument
• near complete absence of hypodermal fat layer at 14 days
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