摘要
用α-甲基苯丙氨酸和苯丙氨酸注射哺乳期大鼠,复制出苯酮尿症动物模型。模型鼠21日龄时,血、脑苯丙氨酸明显增高;脑内酪氨酸减少,氨基酸谱发生严重紊乱,特别是中性氨基酸;脑湿重及脑蛋白总量降低;脑内去甲肾上腺素、多巴胺减少。14～16周龄时,血、脑游离氨基酸、脑蛋白总量趋正常,但'Y'迷宫的学习和记忆能力差,水迷宫发生错误次数增多。提示新生期脑内生化异常,可能引起脑结构异常,并遗留下永久性损害。在治疗苯酮尿症时,除控制苯丙氨酸摄入外,还需补充一些必需氨基酸,可能对预防智能发育障碍有益。
In this study, α-methylphenylalanine plus phenylalanine are administered to suckling rats to reproduce the animal model of phenylketonuria (PKU).When the experimental rats were 21-day-old, phenylalanine content in blood and brain was significantly increased; tyrosine content in brain was decreased. The free amino acid pattern of brain homogenate was significantly disordered and neutral amino acids of the pattern were most obviously reduced in their content. Total protein and wet weight of brain were also reduced. The content of NE and DA in brain was decreased. When the experimental rats were 14-to-16-week-old, there were no differences in total protein content of brain and in free amino acid contents in blood and brain, but abilities to learn and remenber in Y-maze and water-maze of PKU rats remained lower.These results suggest that abnormality of biochemical metabolism in brain during the new born stage may result in structure abnormality of brain, producing permanent damage. The supply of necessary amino acids is benificial to the patients with PKU in prevention of mental retardation, but they must control the intake of phenylalanine in the course of treatment.
出处
《南通医学院学报》
1990年第4期267-270,347-348,共4页
ACTA Academiae Medicinae Nantong
关键词
苯酮尿症
发病机理
氨基酸
记忆
phenylketonuria
amino acid
neuronic transmitter
learn
remenbrance
