苯丙酮酸对大鼠脑内丙酮酸代谢的影响。
The effect of phenylpyruvate on pyruvate metabolism in rat brain.
作者信息
Patel M S
出版信息
Biochem J. 1972 Jul;128(3):677-84. doi: 10.1042/bj1280677.
Abstract
- The effect of phenylalanine and phenylpyruvate on the metabolism of pyruvate by isolated mitochondria from rat brain was investigated. 2. Phenylpyruvate inhibited the fixation of H(14)CO(3) (-) in the presence of pyruvate by intact rat brain mitochondria, whereas phenylalanine and other metabolites of this amino acid had no inhibitory effect on this process. 3. Pyruvate carboxylase activity in freeze-dried rat brain mitochondrial preparations was also inhibited only by phenylpyruvate, and a ;mixed type' inhibition was observed. 4. The K(m) for pyruvate of rat brain pyruvate carboxylase was about 0.2mm. 5. The concentration of phenylpyruvate required for a 50% inhibition of H(14)CO(3) (-) fixation by the intact mitochondria and of pyruvate carboxylase activity was dependent on the concentration of pyruvate used in the incubation medium. 6. The possible significance of inhibition of pyruvate carboxylase activity by phenylpyruvate in the brains of phenylketonuric patients is discussed.
摘要
- 研究了苯丙氨酸和苯丙酮酸对大鼠脑分离线粒体丙酮酸代谢的影响。2. 苯丙酮酸在丙酮酸存在的情况下抑制完整大鼠脑线粒体对H(14)CO(3) (-)的固定，而苯丙氨酸和该氨基酸的其他代谢产物对该过程无抑制作用。3. 冻干大鼠脑线粒体制剂中的丙酮酸羧化酶活性也仅被苯丙酮酸抑制，并观察到“混合型”抑制。4. 大鼠脑丙酮酸羧化酶对丙酮酸的K(m)约为0.2mm。5. 完整线粒体对H(14)CO(3) (-)固定和丙酮酸羧化酶活性50%抑制所需的苯丙酮酸浓度取决于孵育培养基中使用的丙酮酸浓度。6. 讨论了苯丙酮酸抑制苯丙酮尿症患者脑中丙酮酸羧化酶活性的可能意义。
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