Hendriks L, Van Broeckhoven C
Born-Bunge Foundation, University of Antwerp, Flemish Institute of Biotechnology, Department of Biochemistry, Belgium.
Eur J Biochem. 1996 Apr 1;237(1):6-15. doi: 10.1111/j.1432-1033.1996.0006n.x.
Alzheimer's disease is a senile dementia caused by progressive neurodegeneration of the central nervous system. One of the most prominent pathological characteristics is beta A4 amyloid deposition in senile plaques in the brain parenchyma and in cerebral blood vessels. beta A4 amyloid is processed from a larger integral membrane protein, the beta A4 amyloid precursor protein. Different pathogenic mutations in this protein have been detected in a small number of Alzheimer's disease families. Here functional implications of these mutations on the processing of the precursor protein and the beta A4 amyloid deposition will be discussed with respect to the pathogenesis of Alzheimer's disease and related disorders.
阿尔茨海默病是一种由中枢神经系统进行性神经退行性变引起的老年痴呆症。最显著的病理特征之一是β - A4淀粉样蛋白在脑实质和脑血管的老年斑中沉积。β - A4淀粉样蛋白是由一种更大的整合膜蛋白——β - A4淀粉样前体蛋白加工而来。在少数阿尔茨海默病家族中已检测到该蛋白的不同致病突变。本文将结合阿尔茨海默病及相关疾病的发病机制，讨论这些突变对前体蛋白加工及β - A4淀粉样蛋白沉积的功能影响。
