Grignon Y, Colle M A, Hauw J J
Laboratoire de neuropathologie, Raymond-Escourolle, Université Pierre et Marie Curie (Paris VI), INSERM U 360, Association Claude-Bernard, Paris.
Rev Prat. 1997 Oct 15;47(16):1793-7.
Cerebral amyloidoses affects only the central nervous system, with rare exceptions. Most of them are related to A beta deposits. They usually occur in the absence of genetic defect in the precursor of A beta. The prevalence and density of A beta deposits increase during the aging process, and in Alzheimer's disease. This A beta amyloidosis has never been transmitted. In contrast, PrPres occurs as a sporadic or genetic event, and induces transmissible amyloidoses (Creutzfeldt-Jakob's disease and other disorders related to non conventional agents). PrPres may be the infectious agent itself (prion hypothesis). Other proteins are rarely responsible for cerebral amyloidoses. The fascinating hypothesis that a common mechanism would be acting in all cerebral amyloidoses has not yet been confirmed.
脑淀粉样变性仅累及中枢神经系统,罕见例外情况。其中大多数与β淀粉样蛋白沉积有关。它们通常在β淀粉样蛋白前体无基因缺陷的情况下发生。β淀粉样蛋白沉积的患病率和密度在衰老过程以及阿尔茨海默病中会增加。这种β淀粉样变性从未有过传播。相比之下,异常朊蛋白以散发性或遗传性事件的形式出现,并引发可传播的淀粉样变性(克雅氏病及其他与非常规病原体相关的疾病)。异常朊蛋白可能本身就是感染因子(朊病毒假说)。其他蛋白质很少导致脑淀粉样变性。关于一种共同机制在所有脑淀粉样变性中起作用这一引人入胜的假说尚未得到证实。