Suzuki A, Takashima S, Mizuguchi M, Kato M, Kunishita T, Tabira T
Department of Mental Retardation and Birth Defect Research, National Center of Neurology and Psychiatry, Tokyo.
Tohoku J Exp Med. 1994 Nov;174(3):181-7. doi: 10.1620/tjem.174.181.
Down syndrome (DS) brains, from 19 gestational weeks to 50 years of age were studied by immunohistochemical methods with a polyclonal antibody against synthetic peptide comprising part of the Kunitz-type protease inhibitor (KPI) domain of Alzheimer disease amyloid precursor protein (APP), residues 301 to 323 of APP 770. In DS, positive KPI immunoreactivity was observed in early infancy and from child to adulthood on the tunica media of the arteries in the leptomeninges, cerebral cortex and white matter, but negative or little in controls. In DS with Alzheimer type dementia, KPI immunoreactivity in the arteries was reduced, but a gross granular reactivity was noted in neurons and glial cells. The high expression of KPI in DS vessels may be one of the predisposing factors to vascular diseases and amyloid deposition associated with DS.
采用针对包含阿尔茨海默病淀粉样前体蛋白（APP）770的库尼茨型蛋白酶抑制剂（KPI）结构域一部分的合成肽（APP 770的301至323位残基）的多克隆抗体，通过免疫组化方法研究了孕19周龄至50岁的唐氏综合征（DS）患者的大脑。在DS患者中，在婴儿早期以及儿童至成人阶段，在软脑膜、大脑皮质和白质的动脉中膜均观察到KPI免疫反应阳性，但在对照组中为阴性或弱阳性。在患有阿尔茨海默型痴呆的DS患者中，动脉中的KPI免疫反应性降低，但在神经元和神经胶质细胞中观察到明显的颗粒状反应。DS血管中KPI的高表达可能是与DS相关的血管疾病和淀粉样蛋白沉积的易感因素之一。
