Andersen A E, Rowe V, Guroff G
Proc Natl Acad Sci U S A. 1974 Jan;71(1):21-5. doi: 10.1073/pnas.71.1.21.
The biochemical features of phenylketonuria have been reproduced in developing rat pups by administering to them a combination of p-chloro-DL-phenylalanine plus L-phenylalanine for the first 21 days after birth. During the treatment period, the experimental animals show delayed eye opening and decreased brain weight compared with controls given saline. Neuropathological examination of developing animals reveals deficient myelination and some inhibition of cerebellar maturation. When tested as adults, after a long recovery period, animals with phenylketonuria are hyperactive in activity wheels. Adult rats are deficient in reversing a position choice and demonstrate impaired performance in a Y-maze. Rats treated with p-chloro-DL-phenylalanine plus L-phenylalanine during the vulnerable period of rapid brain development thus have enduring behavioral changes that persist throughout life.
通过在出生后的前21天给发育中的幼鼠喂食对氯-DL-苯丙氨酸和L-苯丙氨酸的组合，已在幼鼠中重现了苯丙酮尿症的生化特征。在治疗期间，与给予生理盐水的对照组相比，实验动物的睁眼延迟且脑重量减轻。对发育中动物的神经病理学检查显示髓鞘形成不足以及小脑成熟受到一定抑制。在经过长时间恢复期后作为成年动物进行测试时，患有苯丙酮尿症的动物在活动轮中表现出多动。成年大鼠在反转位置选择方面存在缺陷，并且在Y迷宫中表现受损。因此，在快速脑发育的脆弱期用对氯-DL-苯丙氨酸和L-苯丙氨酸治疗的大鼠具有持续一生的行为改变。
