Avins L, Guroff G, Kuwabara T
J Neuropathol Exp Neurol. 1975 Mar;34(2):178-88. doi: 10.1097/00005072-197503000-00006.
The morphologic effects of hyperphenylalaninemia induced by treatment with para-chlorophenylalanine (PCP) plus phenylalanine on optic nerve were studied in developing F344 rats. PCP and phenylalanine were infected daily between days 5 and 20 days of life. At 20 days optic nerve of treated animals, as compared with saline-injected controls, showed enhanced neuroglial activity with broad astrocytic septae and debris-laden oligodendrocytes. In specimens obtained long after treatment with PCP and phenylalanine, continuing gliosis with evidence of focally abnormal myelination and axonal degeneration were observed. The results are consistent with a metabolic insult sustained in early development by astrocytes and oligodendrocytes, and are considered in relation to other work in experimental hyperphenylalaninemia and to human phenylketonuria.
在发育中的F344大鼠中，研究了用对氯苯丙氨酸（PCP）加苯丙氨酸处理诱导的高苯丙氨酸血症对视神经的形态学影响。在出生后第5天至第20天期间，每天给大鼠注射PCP和苯丙氨酸。在第20天时，与注射生理盐水的对照组相比，接受处理的动物的视神经显示神经胶质细胞活性增强，有宽阔的星形胶质细胞间隔和充满碎片的少突胶质细胞。在用PCP和苯丙氨酸处理很长时间后获得的标本中，观察到持续的胶质增生，有局灶性异常髓鞘形成和轴突变性的证据。这些结果与星形胶质细胞和少突胶质细胞在早期发育中遭受的代谢损伤一致，并与实验性高苯丙氨酸血症的其他研究以及人类苯丙酮尿症相关联进行了考虑。
