苯酮尿症(phenylketonuria,简称PKU)是一种人类先天性代谢缺陷病,主要表现是肝脏内苯丙氨酸羟化酶缺如或不足,导致血液和组织中苯丙氨酸含量增高和酪氨酸含量减少,由此而产生一系列代谢改变(包括尿中苯酮酸等增多),导致严重的智能发育障碍甚致白痴。因而是世界范围内遗传病研究的重点。在我国,它也是计划生育和优生工作中重点防洽的疾病之一。为了深入研究该病的发病机制和实验治疗,需要建立该病的动物模型。迄今为止,尚无该病的自发模型。因而,学者们纷纷探寻诱发性模型。我们参考国外文献,用α-甲基苯丙氨酸作用于大鼠,试图产生实验性苯酮尿症,其目的是为了在国内复制出实验性苯酮尿症模型,为同行深入研究该病提供有用的工具。 Phenylketonuria (phenylketonuria, PKU for short) is a human congenital metabolic defect characterized mainly by a deficiency or deficiency of phenylalanine hydroxylase in the liver, resulting in an increase in the phenylalanine content in the blood and tissues, As a result, a series of metabolic changes (including the increase of uronic acids such as phenylketon acid) lead to serious idiosyncratic disorders. It is therefore the focus of genetic disease research worldwide. In our country, it is also one of the key preventive measures in family planning and eugenics work. In order to further study the pathogenesis of the disease and experimental treatment, we need to establish the animal model of the disease. So far, there is no spontaneous model of the disease. Therefore, scholars have to explore the induced model. We refer to foreign literature, with α-methyl-phenylalanine role in rats, trying to produce experimental phenylketonuria, its purpose is to replicate in the country experimental model of phenylketonuria for colleagues in-depth study of the disease Provide useful tools