Kuchel P W, Roberts D V, Nichol L W
Aust J Exp Biol Med Sci. 1977 Jun;55(3):309-26. doi: 10.1038/icb.1977.26.
Steady-state rate equations are written on the basis of information obtained from the literature describing the kinetics of the four enzyme-catalysed reactions comprising the urea cycle. These equations are formulated into a set which also accounts for fluxes of input and output compounds external to the cycle. Numerical integration of this set of equations is performed employing parameters selected to approximate those pertaining to the operation of the urea cycle in normal liver. The result is a pattern of intermediate metabolite concentrations, which forms a basis for the comparison of patterns reflecting the effects of inborn errors of metabolism. The latter are calculated by varying specified kinetic parameters in the numerical integration. Each of the observed clinical syndromes. Hyperammonemia Types I and II, Hyperarginemia, Citrullinemia and Argininosuccinicaciduria, is discussed.
稳态速率方程是根据从文献中获得的信息编写的，这些文献描述了构成尿素循环的四个酶催化反应的动力学。这些方程被编制成一组，该组方程还考虑了循环外部输入和输出化合物的通量。使用选择的参数对这组方程进行数值积分，这些参数近似于正常肝脏中尿素循环运行的相关参数。结果是中间代谢物浓度的模式，这为比较反映先天性代谢缺陷影响的模式奠定了基础。后者是通过在数值积分中改变特定的动力学参数来计算的。文中讨论了观察到的每种临床综合征，即I型和II型高氨血症、高精氨酸血症、瓜氨酸血症和精氨琥珀酸尿症。
