DiMarco J P, Hoppel C
J Clin Invest. 1975 Jun;55(6):1237-44. doi: 10.1172/JCI108042.
The study was designed to evaluate hepatic mitochondrial function during ketotic states. The ketogenic models studied were streptozotocin-induced diabetic ketoacidosis, 48 h of starvation, and after growth hormone administration. In the last-mentioned model we observed increased free fatty acids but not ketonemia. Oxidative phosphorylation was measured using the citric acid cycle substrates pyruvate and succinate, the amino acid glutamate, a ketone body beta-hydroxybutyrate, and a long-chain fatty acid palmitoyl-l-carnitine. State 3 (ADP stimulated) and state 4 (ADP limited) respiration, respiratory control ratio (state 3/state 4), and the ADP/O ratios were normal in the controls and the experimental groups. Uncoupled respiration produced by dinitrophenol with a variety of substrates was unchanged in the experimental groups compared to the controls. Fatty acid oxidation was studied in detail. The rate of utilization of palmitoyl-l-carnitine by controls or experimental groups did not depend on the product formed (citrate, acetoacetate). No significant changes were observed in the oxidation of palmitoyl-CoA (+ carnitine) or with an intermediate-chain fatty acid hexanoate. The specific activity of hepatic mitochondria carnitine palmitoyltransferase did not change in any of the three experimental groups. It is concluded that during diabetic ketoacidosis, starvation, and growth hormone administration, there is (a) no alteration in hepatic mitochondrial function; (b) no change in the intrinsic capacity of hepatic mitochondria to oxidize fatty acids; and (c) no change in the specific activity of mitochondrial carnitine palmitoyltransferase. The mechanism by which the body restrains flux through the mitochondrial oxidative machinery remains to be fully determined.
该研究旨在评估酮症状态下的肝脏线粒体功能。所研究的生酮模型包括链脲佐菌素诱导的糖尿病酮症酸中毒、48小时饥饿以及生长激素给药后。在最后提到的模型中,我们观察到游离脂肪酸增加,但酮血症未增加。使用柠檬酸循环底物丙酮酸和琥珀酸、氨基酸谷氨酸、酮体β-羟基丁酸以及长链脂肪酸棕榈酰-L-肉碱来测量氧化磷酸化。对照组和实验组的状态3(ADP刺激)和状态4(ADP受限)呼吸、呼吸控制率(状态3/状态4)以及ADP/O比值均正常。与对照组相比,实验组中由二硝基苯酚与多种底物产生的解偶联呼吸没有变化。对脂肪酸氧化进行了详细研究。对照组或实验组对棕榈酰-L-肉碱的利用速率不取决于所形成的产物(柠檬酸、乙酰乙酸)。在棕榈酰辅酶A(+肉碱)或中链脂肪酸己酸的氧化过程中未观察到显著变化。在三个实验组中的任何一组中,肝脏线粒体肉碱棕榈酰转移酶的比活性均未改变。得出的结论是,在糖尿病酮症酸中毒、饥饿和生长激素给药期间,(a)肝脏线粒体功能无改变;(b)肝脏线粒体氧化脂肪酸的内在能力无变化;(c)线粒体肉碱棕榈酰转移酶的比活性无变化。身体限制通过线粒体氧化机制的通量的机制仍有待充分确定。