catabolismoffattyacids2资料.ppt

习题 一分子硬脂酸彻底降解可生成多少ATP？经历哪些代谢途径？写出总反应方程式。 在脂肪酸β－氧化的过程与柠檬酸循环中的部分反应过程类似。试写出这两个途径中的类似的反应过程。 糖尿病患者一般都患有严重酮病。如果给她服用14C标记的乙酰CoA（乙酰基的两个碳都标记），那么她呼出的气体中是否含有14C标记的丙酮？说明理由。 * * Fatty acyl-CoA is formed from fatty acids and coenzymeA via a fatty acyl-AMP intermediate 活化的脂酰辅酶A由肉碱带进线粒体 The fatty acyl group is attached to carnitine (肉碱） via transesterification by the action of carnitine acyltransferase I located on the outer face of the inner membrane, forming fatty acyl-carnitine, leaving the CoA in the cytosol. The acyl carnitine/carnitine transporter moves acyl-carnitine across the inner membrane of mitochondria via facilitated diffusion(易化扩散). Medium-chain acyl-CoAs seem to enter the matrix by themselves, without being carried by carnitine. The acyl group is then transferred back to CoA to form fatty acyl-CoA by the action of carnitine acyltransferase II located on the inner face of the inner membrane. This entering step seems to be rate-limiting for fatty acid oxidation in mitochondria and disease have been found to be caused by a defect of this step (with aching muscle cramp(抽筋), especially during fasting, exercise or when on a high-fat diet). 脂酰辅酶A通过?-氧化生成乙酰辅酶A The b oxidation consists of four reactions: oxidation by FAD, hydration, oxidation by NAD+, and thiolysis by CoA (mainly revealed by David Green, Severo Ochoa, and Feodor Lynen). The 1st oxidation is catalyzed by the membrane-bound acyl-CoA dehydrogenase, converting acyl-CoA to trans-?2-enoyl-CoA with electrons collected by FAD and further transferred into the respiratory chain via the electron-transferring flavoprotein (ETFP), also bound to the inner membrane of the mitochondria. Acyl-CoA dehydrogenase exists in three isozymes acting on the acyl-CoAs of long (12-18C), medium (4-14C) and short (4-8C) chains. The hydration step, stereospecifically catalyzed by enoyl-CoA hydratase, converts the trans-?2-enoyl-CoA to L-b-hydroxylacyl-CoA (the cis isomer can also be converted, but to the D isomer). The second oxidation