Simpson D P, Hager S R
J Clin Invest. 1979 Apr;63(4):704-12. doi: 10.1172/JCI109353.
Mitochondria from rabbit and dog renal cortex were incubated with 1 mM (14)C-weak acid anions in media containing low (10 mM) or high (40 mM) concentrations of bicarbonate and the steady-state accumulation of labeled anion in the matrix was measured. In the absence of an energy source, no concentration of (14)C-anion in the mitochondrial matrix space was present, but the anion concentration was significantly higher at low- than at high-bicarbonate concentration. Addition of an energy source, usually ascorbate plus tetramethyl-p-phenylenediamine, led to increases in matrix space anion levels and to accentuation of the difference in anion uptake between low- and high-bicarbonate media, so that two to four times as much anion was present at low- than at high-bicarbonate concentrations. The anions affected included substrates for which inner membrane carriers are present in mitochondria, such as citrate, alpha-ketoglutarate, malate, and glutamate, as well as substances which diffuse passively across the inner membrane such as acetate and formate. When a nonbicarbonate medium buffered with Hepes was used, pH change did not alter anion uptake although anion concentrations exceeding those in the medium still developed when an energy source was present. The difference in mitochondrial anion accumulation between low- and high-bicarbonate levels diminished with decreasing temperature or with increasing anion concentration in the medium. Estimation of intramitochondrial pH with [(14)C]5,5-dimethyl-oxazolidine-2,4-dione showed that the pH gradient across the inner mitochondrial membrane was significantly greater with 10 than with 40 mM bicarbonate in the medium.A hypothesis is described that relates this effect of pH and bicarbonate on mitochondrial anion accumulation to the very rapid changes in substrate levels in renal cortex, which develop when acute metabolic acidosis or alkalosis is produced in the intact animal. It is suggested that an abrupt fall in systemic pH and bicarbonate is associated with a shift in substrate in renal cortex out of the cytoplasm and into mitochondria, where some of the added substrate is metabolized. Reduction in the size of the cytoplasmic pool of substrate occurs with relatively little accompanying change in the size of the mitochondrial pool, thus causing a net reduction in the total tissue pool. This mechanism accounts for the reduction in tissue levels of many mitochondrial substrates observed acutely in metabolic acidosis. In metabolic alkalosis, reversal of these effects leads to expansion of the cytoplasmic pool, thereby resulting in the rise in tissue levels of substrates which occurs in this condition.
将来自兔和犬肾皮质的线粒体与含低浓度(10 mM)或高浓度(40 mM)碳酸氢盐的培养基中的1 mM (14)C-弱酸阴离子一起孵育,并测量标记阴离子在基质中的稳态积累。在没有能量来源的情况下,线粒体基质空间中不存在(14)C-阴离子的浓度,但在低碳酸氢盐浓度下阴离子浓度显著高于高碳酸氢盐浓度。添加能量来源,通常是抗坏血酸加四甲基对苯二胺,会导致基质空间阴离子水平升高,并加剧低碳酸氢盐和高碳酸氢盐培养基之间阴离子摄取的差异,因此在低碳酸氢盐浓度下存在的阴离子是高碳酸氢盐浓度下的两到四倍。受影响的阴离子包括线粒体中存在内膜载体的底物,如柠檬酸、α-酮戊二酸、苹果酸和谷氨酸,以及被动扩散穿过内膜的物质,如乙酸盐和甲酸盐。当使用用Hepes缓冲的非碳酸氢盐培养基时,pH变化不会改变阴离子摄取,尽管当存在能量来源时,阴离子浓度仍会超过培养基中的浓度。随着温度降低或培养基中阴离子浓度增加,低碳酸氢盐和高碳酸氢盐水平之间线粒体阴离子积累的差异减小。用[(14)C]5,5-二甲基-恶唑烷-2,4-二酮估计线粒体内pH表明,当培养基中碳酸氢盐为10 mM时,跨线粒体内膜的pH梯度显著大于40 mM时。描述了一个假设,将pH和碳酸氢盐对线粒体阴离子积累的这种影响与肾皮质中底物水平的非常快速的变化联系起来,当在完整动物中产生急性代谢性酸中毒或碱中毒时就会出现这种变化。有人提出,全身pH和碳酸氢盐的突然下降与肾皮质中底物从细胞质转移到线粒体有关,在那里一些添加的底物被代谢。底物的细胞质池大小减少,而线粒体池大小的伴随变化相对较小,从而导致总组织池净减少。这种机制解释了在代谢性酸中毒中急性观察到的许多线粒体底物组织水平的降低。在代谢性碱中毒中,这些效应的逆转导致细胞质池扩大,从而导致在这种情况下出现的底物组织水平升高。