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• W2022050718 abstract "Juvenile visceral steatosis (JVS) mice have been reported to have systemic carnitine deficiency, and the carnitine concentration in the liver of JVS mice was markedly lower than that of controls (11.6 ± 2.6 versus 393.5 ± 56.4 nmol/g of wet liver). To evaluate the role of carnitine in mitochondrial β-oxidation in liver, we examined the effects of carnitine on ketogenesis in perfused liver from control and JVS mice. In control mice, ketogenesis was increased by the infusion of 0.3 mM oleate, but not by L-carnitine. In contrast, although ketogenesis in JVS mice was not increased by the infusion of oleate, it was increased 2.5-fold by the addition of 1000μM L-carnitine. Addition of 50, 100, and 200 μM L-carnitine increased ketogenesis in a dose-dependent manner. The infusion of 0.3 mM octanoate or butyrate increased ketogenesis in a carnitine-independent fashion in both control and JVS mice. These findings suggest that endogenous long chain fatty acids from accumulated triglycerides may be used as substrates in the presence of carnitine in JVS mice. The relationship between ketogenesis and free carnitine concentration was examined in livers from JVS mice. Ketogenesis increased as free carnitine levels increased until concentrations exceeded about 100 nmol/g of wet liver (340 μM). The free carnitine concentration required for half-maximal ketone body production in liver of JVS mice was 45μM (13 nmol/g of wet liver), which corresponds to a Km value of carnitine palmitoyltransferase I. We conclude that carnitine is a rate-limiting factor for β-oxidation in liver only when the carnitine level in liver is very low." @default.
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• W2022050718 date "1997-07-01" @default.
• W2022050718 modified "2023-10-12" @default.
• W2022050718 title "The Effect of Carnitine on Ketogenesis in Perfused Livers from Juvenile Visceral Steatosis Mice with Systemic Carnitine Deficiency" @default.
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• W2022050718 doi "https://doi.org/10.1203/00006450-199707000-00017" @default.
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