Analysis Tools
cardiovascular system
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• short-, medium- and long-chain acylcarnitine contents in the heart are decreased by 86%, 63% and 82%, respectively
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• cardiac muscle cells exhibit increased number of small mitochondria and a decreased percentage of large mitochondria
• however, citrate synthase activity, amount of mitochondrial DNA, and expression level of mitochondrial biogenesis and fission- and fusion-related genes are normal
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• carnitine-independent CPT2-coupled beta-oxidation of long-chain acylcarnitines as well as the pyruvate (N) or pyruvate and succinate (NS) pathway-linked respiration rate in the OXPHOS state is 24-46% higher in cardiac fibers
• however, no difference in fatty acid oxidation-dependent and NS pathway-dependent OXPHOS coupling efficiency or mitochondrial ROS production under normoxic conditions is seen
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• N- and NS-linked oxidative phosphorylation (OXPHOS)-dependent respiration rates and OXPHOS coupling efficiency are preserved under ischemia-reperfusion damage and the H2O2/O ratio is lower in the fatty acid oxidation-, N-, and NS-linked OXPHOS state indicating that cardiac mitochondrial functionality is protected from ischemia-reperfusion induced damage
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• infract size is smaller in response to myocardial ischemia-reperfusion
• however, heart functional parameters of isolated Langendorff-perfused hearts are similar to wild-type mice and cardiac flow, cardiac work, LVDP, and heart rate are normal during occlusion and reperfusion
• however, heart function is normal: ejection fraction and fractional shortening of the left ventricle are normal and no differences in the thicknesses of the interventricular septum at end-systole, interventricular septum at end-diastole, and left ventricular posterior wall at end-diastole are seen and in Langendorff-perfused isolated hearts, the functional parameters coronary flow, left ventricular developed pressure, heart rate and contractility are normal
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cellular
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• carnitine-independent CPT2-coupled beta-oxidation of long-chain acylcarnitines as well as the pyruvate (N) or pyruvate and succinate (NS) pathway-linked respiration rate in the OXPHOS state is 24-46% higher in cardiac fibers, indicating that mitochondrial bioenergetics related to pyruvate and fatty acid metabolism are stimulated
• 25% lower lactate and succinate concentrations in the plasma, indicating that mitochondrial metabolism of lactate/pyruvate and the Krebs cycle are increased in response to the lower long-chain acylcarnitine levels
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• fatty acid oxidation rate in isolated intact hearts is decreased by 35%
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homeostasis/metabolism
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• N- and NS-linked oxidative phosphorylation (OXPHOS)-dependent respiration rates and OXPHOS coupling efficiency are preserved under ischemia-reperfusion damage and the H2O2/O ratio is lower in the fatty acid oxidation-, N-, and NS-linked OXPHOS state indicating that cardiac mitochondrial functionality is protected from ischemia-reperfusion induced damage
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• infract size is smaller in response to myocardial ischemia-reperfusion
• however, heart functional parameters of isolated Langendorff-perfused hearts are similar to wild-type mice and cardiac flow, cardiac work, LVDP, and heart rate are normal during occlusion and reperfusion
• however, heart function is normal: ejection fraction and fractional shortening of the left ventricle are normal and no differences in the thicknesses of the interventricular septum at end-systole, interventricular septum at end-diastole, and left ventricular posterior wall at end-diastole are seen and in Langendorff-perfused isolated hearts, the functional parameters coronary flow, left ventricular developed pressure, heart rate and contractility are normal
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• fatty acid oxidation rate in isolated intact hearts is decreased by 35%
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• 90% lower concentrations of gamma-butyrobetaine and carnitine in plasma
• however, levels of plasma glucose, fatty acids and triglycerides in the plasma and liver are normal
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• 25% lower lactate concentrations in the plasma
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• lipids mostly consisting of saturated or monounsaturated fatty acids are decreased by up to 40% in plasma, while lipids consisting of more than 6 double bonds are increased by up to 80%
• however, the total concentrations of lysophosphatidylcholine, phosphatidyl-cholines, sphingomyelins, ceramides, dihexosyl-ceramides, and cholesteryl esters are normal
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• plasma concentrations of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) are increased by approximately 35% in fed mice and by approximately 2.2- and 1.5-fold higher in the fasted state
• however, concentrations of palmitate and other non-esterified saturated fatty acids in the plasma are normal
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• the lipid profile is markedly shifted to a significantly higher abundance of unsaturated fatty acids
• increase in concentrations of polyunsaturated fatty acids in the plasma
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muscle
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• cardiac muscle cells exhibit increased number of small mitochondria and a decreased percentage of large mitochondria
• however, citrate synthase activity, amount of mitochondrial DNA, and expression level of mitochondrial biogenesis and fission- and fusion-related genes are normal
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behavior/neurological
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• adult and aged mice show normal muscular coordination ad motor function
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