Kaplan H, Triano T, Donadio M
Dev Psychobiol. 1981 May;14(3):201-7. doi: 10.1002/dev.420140308.
To investigate the relationship between the biochemical and behavioral deficits in phenylketonuria (PKU), we treated rats from Postnatal Days 2 through 21 with p-chlorophenylalanine plus L-phenylalanine to simulate PKU or with one of the following aromatic acid metabolites of phenylalanine phenylacetate, phenylpyruvate, phenyllactate, and mandelate. Behavioral tests were begun at about 9 weeks of age. Differences between experimental and control animals were found only in the water maze and in the open field. In the former, PKU rats required significantly more trials to reach criterion than controls. None of the single metabolite-treated groups exhibited a similar learning deficit. In the open field, PKU and mandelate-treated rats were hypoactive compared with controls, whereas phenylacetate- and phenylpyruvate-treated rats wee hyperactive. These results demonstrate a lack of congruence between morphological and behavioral effects of treatment, suggesting that performance deficits in PKU rats may be due to interactive effects of 2 or more metabolites.
为了研究苯丙酮尿症（PKU）患者生化指标与行为缺陷之间的关系，我们从出生后第2天至第21天用对氯苯丙氨酸加L-苯丙氨酸处理大鼠，以模拟PKU，或用苯丙氨酸的以下芳香酸代谢产物之一苯乙酸、苯丙酮酸、苯乳酸和扁桃酸处理大鼠。行为测试在大约9周龄时开始。仅在水迷宫和旷场试验中发现实验动物与对照动物之间存在差异。在水迷宫试验中，PKU大鼠达到标准所需的试验次数明显多于对照组。单一代谢产物处理组均未表现出类似的学习缺陷。在旷场试验中，与对照组相比，PKU大鼠和扁桃酸处理的大鼠活动减少，而苯乙酸和苯丙酮酸处理的大鼠活动增加。这些结果表明治疗的形态学和行为学效应之间缺乏一致性，提示PKU大鼠的行为缺陷可能是由于两种或更多种代谢产物的相互作用所致。
