Neuropathology of phenylacetate poisoning in rats: An experimental model of phenylketonuria
Neuropathology of phenylacetate poisoning in rats: An experimental model of phenylketonuria
复制标题
大鼠苯乙酸中毒的神经病理学：苯丙酮尿症的实验模型
作者：
PhD G. Y . Wen;MD H. M. Wisniewski;J. W. S. PhD;MD Y. H. Loo;PhD T. R. Fulton;D. Wen
本研究结果表明，苯乙酸处理的乳鼠是研究苯丙酮尿症发病机制和神经元发育的一种有用的新模型。出生后处理的大鼠小脑和视网膜神经元都容易受到苯乙酸酯的不良影响。在出生后第4 - 21天，在给药动物的小脑、视网膜和视神经中观察到的形态学变化包括小脑蚓部小叶IV、V、VIa和IX的尺寸局部减小，分子层厚度减少35 - 40%，小脑外颗粒细胞和视网膜成神经细胞蓄积，小脑皮质中平行纤维减少，视神经中有髓鞘的轴突更少。
Results of this investigation indicate that the suckling rat treated with phenylacetate should be a useful new model for studying the pathogenesis of phenylketonuria and neuronal development. Both cerebellar and retinal neurons of postnatally treated rats are vulnerable to the adverse effects of phenylacetate. Morphological changes observed in the cerebellum, retina, and optic nerve of treated animals during the fourth to twenty‐first days of life consist of regional reduction in the size of cerebellar vermis lobules IV, V, VIa, and IX, 35 to 40% reduction in thickness of the molecular layer, accumulation of cerebellar external granular cells and retinal neuroblastic cells, fewer parallel fibers in the cerebellar cortex, and fewer myelinated axons in the optic nerve.