对氯苯丙氨酸诱发大鼠苯丙酮尿症的化学表现

p-Chlorophenylalanine-induced chemical manifestations of phenylketonuria in rats.

作者信息

Lipton M A, Gordon R, Guroff G, Udenfriend S

出版信息

Science. 1967 Apr 14;156(3772):248-50. doi: 10.1126/science.156.3772.248.

DOI:10.1126/science.156.3772.248

Abstract

p-Chlorophenylalanine, a potent inhibitor of phenylalanine hydroxylase in vivo, has been used to simulate phenylketonuria in rats. This inhibitor, when administered with phenylalanine, produces marked elevation of blood and tissue phenylalanine without an increase in tyrosine. Disproportionate ratios of phenylalanine to tyrosine are characteristic of phenylketonuria in humans. The use of p-chlorophenylalanine permits the production of this characteristics amino acid imbalance in experimental animals.

摘要

对氯苯丙氨酸是一种在体内对苯丙氨酸羟化酶有强效抑制作用的物质，已被用于在大鼠中模拟苯丙酮尿症。这种抑制剂与苯丙氨酸一起给药时，会使血液和组织中的苯丙氨酸显著升高，而酪氨酸却不会增加。苯丙氨酸与酪氨酸的比例失调是人类苯丙酮尿症的特征。使用对氯苯丙氨酸能够在实验动物中产生这种特征性的氨基酸失衡。