Casey C A, Anderson P M
Comp Biochem Physiol B. 1985;82(2):307-15. doi: 10.1016/0305-0491(85)90246-9.
The submitochondrial localization of the four mitochondrial enzymes associated with urea synthesis in liver of Squalus acanthias (spiny dogfish), a representative elasmobranch, was determined. Glutamine- and acetylglutamate-dependent carbamoyl-phosphate synthetase, ornithine carbamoyltransferase, glutamine synthetase, and arginase were all localized within the matrix of liver mitochondria. The subcellular and submitochondrial localization and activities of several related enzymes involved in nitrogen metabolism and gluconeogenesis in liver and dogfish are also reported. Pyruvate carboxylase and phosphoenolpyruvate carboxykinase were localized in the mitochondrial matrix. Synthesis of citrulline by isolated mitochondria from ornithine proceeds at a near optimal rate at ornithine concentrations as low as 0.08 mM. The same stoichiometry and rates of citrulline synthesis are observed when ornithine is replaced by arginine. The mitochondrial location of arginase does not appear to reflect a mechanism for regulating ornithine availability.
对一种典型的板鳃亚纲动物——棘鲨(刺鲨)肝脏中与尿素合成相关的四种线粒体酶的亚线粒体定位进行了测定。谷氨酰胺和乙酰谷氨酸依赖性氨甲酰磷酸合成酶、鸟氨酸氨甲酰转移酶、谷氨酰胺合成酶和精氨酸酶均定位于肝脏线粒体基质中。还报道了肝脏和鲨鱼体内参与氮代谢和糖异生的几种相关酶的亚细胞和亚线粒体定位及活性。丙酮酸羧化酶和磷酸烯醇式丙酮酸羧激酶定位于线粒体基质中。从鸟氨酸合成瓜氨酸时,分离的线粒体在低至0.08 mM的鸟氨酸浓度下以接近最佳的速率进行反应。当鸟氨酸被精氨酸取代时,观察到相同的化学计量和瓜氨酸合成速率。精氨酸酶的线粒体定位似乎并不反映调节鸟氨酸可用性的机制。