Proteinopathies/Neurodegenerative Diseases - ZBS6, Robert Koch-Institut, Berlin, Germany.
Department of Neuropathology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Acta Neuropathol Commun. 2018 Mar 5;6(1):23. doi: 10.1186/s40478-018-0511-7.
Seeding and spread of beta-amyloid (Aβ) pathologies have been considered to be based on prion-like mechanisms. However, limited transmissibility of Aβ seeding activity upon peripheral exposure would represent a key difference to prions, not only in terms of pathogenesis but also in terms of potential transmission of disease. We partially characterized the seeded Aβ amyloidosis after intracerebral injection of various brain homogenates in APP/PS1 mice. One particularly seed-laden homogenate was selected to investigate the development of Aβ pathologies after intravenous exposure. We report here that a single intravenous injection of an Alzheimer disease patient's-brain extract into APP/PS1 recipient mice led to cerebral amyloid angiopathy within 180 days post injection. Thus, vascular proteinopathies such as CAA are transmissible in mice via the intravenous route of peripheral exposure.
β-淀粉样蛋白（Aβ）病理学的播种和传播被认为是基于类朊病毒机制。然而，外周暴露时 Aβ 播种活性的有限传染性将代表与朊病毒的一个关键区别，不仅在发病机制方面，而且在疾病的潜在传播方面。我们部分描述了 APP/PS1 小鼠脑匀浆内注射各种脑匀浆后，被播种的 Aβ 淀粉样变性。选择了一个特别载有种子的匀浆来研究静脉暴露后 Aβ 病理学的发展。我们在这里报告，单次静脉注射阿尔茨海默病患者的脑提取物到 APP/PS1 受者小鼠中，导致脑淀粉样血管病在注射后 180 天内发生。因此，血管蛋白病如 CAA 可通过外周暴露的静脉途径在小鼠中传播。
