Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
Prog Mol Biol Transl Sci. 2012;107:41-78. doi: 10.1016/B978-0-12-385883-2.00006-0.
Cerebral amyloid angiopathy (CAA) is cerebrovascular amyloid deposition. It is classified into several types according to the cerebrovascular amyloid proteins involved [amyloid β-protein (Aβ), cystatin C (ACys), prion protein (APrP), transthyretin (ATTR), gelsolin (AGel), ABri/ADan, and AL]. Sporadic Aβ-type CAA is commonly found in elderly individuals and patients with Alzheimer's disease (AD). CAA-related disorders include hemorrhagic and ischemic brain lesions and dementia. It has been proposed that cerebrovascular Aβ originates mainly from the brain and is transported to the vascular wall through a perivascular drainage pathway, where it polymerizes into fibrils on vascular basement membrane through interactions with extracellular components. CAA would be promoted by overproduction of Aβ40 (a major molecular species of cerebrovascular Aβ), a decrease of Aβ degradation, or reduction of Aβ clearance due to impairment of perivascular drainage pathway. Further understanding of the molecular pathogenesis of CAA would lead to development of disease-modifying therapies for CAA and CAA-related disorders.
脑淀粉样血管病（Cerebral amyloid angiopathy，CAA）是脑血管淀粉样物质沉积。根据涉及的脑血管淀粉样蛋白，可将其分为几种类型[β-淀粉样蛋白（Amyloid β-protein，Aβ）、半胱氨酸蛋白酶抑制剂 C（Cystatin C，ACys）、朊病毒蛋白（Prion protein，APrP）、转甲状腺素蛋白（Transthyretin，ATTR）、胶凝蛋白（Gelsolin，AGel）、ABri/ADan 和 AL]。散发性 Aβ 型 CAA 常见于老年人和阿尔茨海默病（Alzheimer's disease，AD）患者。与 CAA 相关的疾病包括出血性和缺血性脑损伤和痴呆。有研究提出，脑血管 Aβ 主要来源于大脑，并通过血管周围引流途径被运送到血管壁，在那里通过与细胞外成分的相互作用在血管基底膜上聚集成原纤维。Aβ40 的过度产生（脑血管 Aβ 的主要分子种类）、Aβ 降解减少或由于血管周围引流途径受损导致 Aβ 清除减少，都会促进 CAA 的发生。进一步了解 CAA 的分子发病机制，将有助于开发 CAA 和 CAA 相关疾病的疾病修饰疗法。
