ALPHA-1-ANTICHYMOTRYPSIN IS ASSOCIATED SOLELY WITH AMYLOID DEPOSITS CONTAINING THE BETA-PROTEIN - AMYLOID AND CELL LOCALIZATION OF ALPHA-1-ANTICHYMOTRYPSIN
ALPHA-1-ANTICHYMOTRYPSIN IS ASSOCIATED SOLELY WITH AMYLOID DEPOSITS CONTAINING THE BETA-PROTEIN - AMYLOID AND CELL LOCALIZATION OF ALPHA-1-ANTICHYMOTRYPSIN
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DOI：
10.1016/0197-4580(90)90045-2
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发表时间：
1990-03-01
影响因子：
4.2
通讯作者：
POTTER, H
中科院分区：
文献类型：
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作者：
ABRAHAM, CR;SHIRAHAMA, T;POTTER, H
我们最近的研究表明，α 1-抗胰凝乳蛋白酶（ACT），一种丝氨酸蛋白酶抑制剂，与β-胰凝乳蛋白酶密切相关。阿尔茨海默氏病、老年人对照组和老年猴脑淀粉样蛋白沉积中的蛋白质，表明抑制剂在淀粉样蛋白沉积中的作用。在本研究中，我们使用免疫组织化学来测试淀粉样蛋白沉积物中ACT的存在，所述淀粉样蛋白沉积物含有作为其主要组分的不同于β-ACT的蛋白质。蛋白在原发性或继发性淀粉样变性、家族性和淀粉样多发性神经病或Creutzfeldt-Jakob病的淀粉样沉积物中未发现ACT（非β-蛋白淀粉样变性），但发现（与β-蛋白）在阿尔茨海默氏病、唐氏综合征、正常衰老和遗传性脑出血伴荷兰起源的淀粉样变性中的作用。这些结果表明ACT与β-SMA的特异性关联。淀粉样蛋白我们接下来检查了抑制剂在正常人脑和各种人类神经病理状态中的分布，以鉴定在脑变性期间表达这种蛋白质的细胞。除了与淀粉样蛋白的关联，ACT免疫反应性也位于神经元或组织损失区域附近的星形胶质细胞中，少数神经元和周细胞中以及脉络丛的上皮中。
Our recent studies demonstrated that .alpha.1-antichymotrypsin (ACT), a serine protease inhibitor, was intimately associated with the .beta.-protein in the brain amyloid deposits of Alzheimer''s disease, aged human controls and aged monkeys, suggesting a role for the inhibitor in the amyloid deposition. In the present study we used immunohistochemistry to test for the presence of ACT in the amyloid deposits which contain, as their major component, a protein different from the .beta.-protein. ACT was not found in the amyloid deposits in primary or secondary amyloidosis, familial and amyloidotic polyneuropathy or Creutzfeldt-Jakob disease (non-.beta.-protein amyloidoses), but was found (together with .beta.-protein) in Alzheimer''s disease, Down''s syndrome, normal aging, and hereditary cerebral hemorrhage with amyloidosis of Dutch origin. These results suggest a specific association of ACT with .beta.-protein amyloid. We next examined the distribution of the inhibitor in normal human brain and in various human neuropathological states in order to identify cells that express this protein during brain degeneration. In addition to its association with amyloid, ACT immunoreactivity was also located in astrocytes near areas of neuronal or tissue loss, in a few neurons and pericytes and in the epithelium of the choroid plexus.