Gimenez C, Benavides J, Sanchez-Rubiales M, Valdivieso F, Mayor F
Mol Cell Biochem. 1977 May 31;16(1):9-16. doi: 10.1007/BF01769833.
The "in vivo" effects of L-phenylalanine on the gluconeogenic pathway in the liver of fasted rats with experimentally induced phenylketonuria-like characteristics have been investigated. Significant increases of the fructose 6-phosphate, glucose 6-phosphate and glucose concentrations were observed. The study of the effect of L-phenylalanine on the cytoplasmic and mitochondrial redox state and energy charge showed an increase in the mitochondrial NAD+/NADH ratio while the energy charge was virtually unchanged. The effects of phenylalanine and its metabolic derivatives (phenylacetate, phenylethylamine, phenyl-lactate, o-hydroxyphenylacetate and phenylpyruvate) on the activity of lactate dehydrogenase (EC 1.1.1.27), malate dehydrogenase (EC 1.1.1.37) and 3-hydroxybutyrate dehydrogenase (EC 1.1.1.30) in rat liver have been also investigated. Phenylpyruvate inhibited the lactate dehydrogenase activity with a Ki of 5.3 mM. Phenylpyruvate also inhibited both the mitochondrial (Ki = 4 mM) and cytoplasmic (Ki = 5 mM) malate dehydrogenase activities. Phenylpyruvate, phenylacetate and o-hydroxyphenylacetate inhibited the 3-hydroxybutyrate dehydrogenase activity with Ki values of 0.7, 6.0 and 9.5 mM respectively.
已对具有实验性诱导的苯丙酮尿症样特征的禁食大鼠肝脏中，L-苯丙氨酸对糖异生途径的“体内”作用进行了研究。观察到6-磷酸果糖、6-磷酸葡萄糖和葡萄糖浓度显著增加。对L-苯丙氨酸对细胞质和线粒体氧化还原状态及能荷的影响研究表明，线粒体NAD⁺/NADH比值增加，而能荷实际上未发生变化。还研究了苯丙氨酸及其代谢衍生物（苯乙酸、苯乙胺、苯乳酸、邻羟基苯乙酸和苯丙酮酸）对大鼠肝脏中乳酸脱氢酶（EC 1.1.1.27）、苹果酸脱氢酶（EC 1.1.1.37）和3-羟基丁酸脱氢酶（EC 1.1.1.30）活性的影响。苯丙酮酸以5.3 mM的Ki抑制乳酸脱氢酶活性。苯丙酮酸还抑制线粒体（Ki = 4 mM）和细胞质（Ki = 5 mM）苹果酸脱氢酶活性。苯丙酮酸、苯乙酸和邻羟基苯乙酸分别以0.7、6.0和9.5 mM的Ki值抑制3-羟基丁酸脱氢酶活性。
