Pathophysiology of amyotrophic lateral ]sclerosi using aipha-tocopherol transfer protein gene knockout mouse.

1)补充维生素E可增加和改善aTTP缺失小鼠脑内MDA和HNE的修饰。2) hne修饰的蛋白为SOD。这种修饰干扰了SOD的自由基清除功能，导致维生素E缺乏引起的氧化应激增强。3) MPTP给药后，aTTP缺失小鼠纹状体多巴胺含量降低，提示帕金森病患者多巴胺减少与氧化应激有关。4) G93ASOD1 TgM与aTTP缺失小鼠的症状发作加快，前角细胞变性更为明显，提示ALS的病理生理受到氧化应激的影响。

1) MDA and HNE modifications in the brain of aTTP null mouse were increased and improved by vitamin E supplementation.2) HNE-modified protein is SOD. This modification disturbed the radical scavenge function of SOD, resulting in enhance the oxidative stress by vitamin E deficiency.3) Dopamine content in the striatum of aTTP null mouse was decreased after administration of MPTP, indicating dopamine decrease in Parkinson disease is related to oxidative stress.4) Onset of symptom in G93ASOD1 TgM crossed with aTTP null mouse is accelerated with more marked degeneration of anterior horn cell, indicating pathophysiology of ALS is affected by oxidative stress,