蓝藻菌/β-N-甲氨基-L-丙氨酸学说在肌萎缩侧索硬化症及其他神经变性病致病机制中的研究进展  

The Cyanobacteria/β-N-methylamino-L-alanine Hypothesis of the Pathogenesis of Amyotrophic Lateral Sclerosis and Other Neurodegenerative Diseases

作　　者：牛琦[1] 丁新生[1] 

机构地区：[1]南京医科大学第一附属医院神经科,210029

出　　处：《中国临床神经科学》2012年第3期307-311,共5页Chinese Journal of Clinical Neurosciences

基　　金：江苏省自然科学基金(编号:BK2007607)

摘　　要：肌萎缩侧索硬化症(ALS)是一种进行性致死性神经变性病,按发病类型分为散发性和遗传性两种。其中,散发性ALS发病率全球相对一致,但在关岛等少数西太平洋地区的人群聚居地发病率有增高现象,且常伴有肌萎缩侧索硬化-帕金森-痴呆叠加症(ALS/PDC)。研究发现寄生在苏铁根部的蓝藻菌产生的非蛋白质氨基酸[β-N-甲氨基-L-丙氨酸(BMAA)]与关岛的ALS/PDC发病有关。易感个体长期暴露在富含BMAA的环境中可导致BMAA在神经蛋白中聚集,产生ALS等迟发性进行性神经变性病。本文就蓝藻菌/BMAA作为神经变性病环境致病毒素的假说及其研究进展综述如下。Amyotrophic lateral sclerosis（ALS） is a progressive fatal neurodegenerative disease.According to heredity,it is divided into sporadic and hereditary subtype.The incidence of sporadic ALS is relatively uniform throughout the world,except for clusters of high incidence among Guam and a few other western Pacific foci.There the disease is often combined with ALS/ Parkinson-dementia complex（PCD）.Research on Guam suggested that a non-protein amino acid,β-N-methylamino-L-alanine（BMAA） which produced by cyanobacteria within in specialized roots of the cycads,be relate to the onset of ALS/PDC.Vulnerable individuals can accumulate BMAA in their proteins after long exposure and trigger latent progressive neural degeneration,such as ALS.This review presented the hypothesis of the cyanobacteria/BMAA as environmental toxin and its new insights.

关 键 词：肌萎缩侧索硬化症 肌萎缩侧索硬化-帕金森-痴呆叠加症 β-N-甲氨基-L-丙氨酸 蓝藻菌 关岛 神经变性病 

分 类 号：R746[医药卫生—神经病学与精神病学]