Buist N R
J Inherit Metab Dis. 1980;3(4):113-6. doi: 10.1007/BF02312544.
The co-existence of a hereditary defect of pyruvate carboxylase activity along with proximal renal tubular acidosis in several patients prompted the following theories: (1) Some of the bicarbonate which is normally reabsorbed from the glomerular filtrate is trapped in the mitochondria by pyruvate carboxylase in the conversion of pyruvate to oxaloacetate. The subsequent conversion of oxaloacetate to phosphoenol pyruvate releases CO2 in the cytosol. (2) The trapping of HCO-3 by pyruvate (or other carboxylases) provides an important route for the recovery of filtered HCO-3. (3) The process of trapping HCO-3 from the glomerular filtrate followed by release of CO2 in the cytosol contributes to the apparently high RQ of kidney, since the CO2 does not originate from a metabolic fuel. (4) Lactate and possibly other fuels are actively taken up by the kidney and are used as energy sources. Diversion of lactate for gluconeogenesis may contribute to the "excess substrate uptake" phenomenon. (5) It is possible that some of the glucose which is synthesized in the cortex is used for glycolysis in the medulla. Conversely, lactate produced in the medulla may be available to the cortex for bicarbonate trapping and thus for gluconeogenesis.
几名患者中丙酮酸羧化酶活性的遗传性缺陷与近端肾小管酸中毒并存,引发了以下理论:(1) 通常从肾小球滤液中重吸收的一些碳酸氢盐在丙酮酸转化为草酰乙酸的过程中被丙酮酸羧化酶截留在线粒体中。随后草酰乙酸转化为磷酸烯醇丙酮酸会在细胞质中释放二氧化碳。(2) 丙酮酸(或其他羧化酶)对HCO-3的截留为滤过的HCO-3的回收提供了一条重要途径。(3) 从肾小球滤液中截留HCO-3,随后在细胞质中释放二氧化碳的过程导致肾脏的呼吸商明显较高,因为二氧化碳并非来自代谢燃料。(4) 乳酸以及可能的其他燃料被肾脏主动摄取并用作能量来源。乳酸转向糖异生可能导致“底物摄取过多”现象。(5) 有可能在皮质中合成的一些葡萄糖用于髓质中的糖酵解。相反,髓质中产生的乳酸可能可供皮质用于碳酸氢盐截留,从而用于糖异生。