Levy Efrat, Jaskolski Mariusz, Grubb Anders
Department of Psychiatry, New York University School of Medicine, and Nathan Kline Institute, Orangeburg 10962, USA.
Brain Pathol. 2006 Jan;16(1):60-70. doi: 10.1111/j.1750-3639.2006.tb00562.x.
A variant of the cysteine protease inhibitor, cystatin C, forms amyloid deposited in the cerebral vasculature of patients with hereditary cerebral hemorrhage with amyloidosis, Icelandic type (HCHWA-I), leading to cerebral hemorrhages early in life. However, cystatin C is also implicated in neuronal degenerative diseases in which it does not form the amyloid protein, such as Alzheimer disease (AD). Accumulating data suggest involvement of cystatin C in the pathogenic processes leading to amyloid deposition in cerebral vasculature and most significantly to cerebral hemorrhage in patients with cerebral amyloid angiopathy (CAA). This review focuses on cell culture and animal models used to study the role of cystatin C in these processes.
半胱氨酸蛋白酶抑制剂胱抑素C的一种变体形成淀粉样蛋白，沉积在冰岛型遗传性脑出血伴淀粉样变性（HCHWA-I）患者的脑血管中，导致患者早年发生脑出血。然而，胱抑素C也与不形成淀粉样蛋白的神经退行性疾病有关，如阿尔茨海默病（AD）。越来越多的数据表明，胱抑素C参与了导致脑血管淀粉样沉积的致病过程，最重要的是参与了脑淀粉样血管病（CAA）患者的脑出血过程。本综述重点关注用于研究胱抑素C在这些过程中作用的细胞培养和动物模型。
