Walser M, Stewart P M
J Inherit Metab Dis. 1981;4(4):177-82. doi: 10.1007/BF02263648.
Recent clinical and experimental evidence on the effects of organic acids in producing or ameliorating hyperammonaemia is reviewed. The importance of hepatic mitochondrial N-acetylglutamate and its precursors, glutamate and acetyl-CoA, in the control of ureagenesis and thus blood ammonia levels is emphasized by recent work. The hypothesis is proposed that protein loads stimulated urea cycle activity via glutamate-induced changes in N-acetylglutamate concentration, while the effects of organic acids on ureagenesis are related in a predictable way to their effects on hepatic concentrations of acetyl-CoA.
本文综述了关于有机酸在产生或改善高氨血症方面作用的近期临床和实验证据。近期研究强调了肝脏线粒体N - 乙酰谷氨酸及其前体谷氨酸和乙酰辅酶A在控制尿素生成从而控制血氨水平方面的重要性。本文提出一个假说：蛋白质负荷通过谷氨酸诱导的N - 乙酰谷氨酸浓度变化刺激尿素循环活性，而有机酸对尿素生成的影响与其对肝脏乙酰辅酶A浓度的影响以一种可预测的方式相关。
