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系统性AA淀粉样变性作为一种朊病毒样疾病。

Systemic AA amyloidosis as a prion-like disorder.

作者信息

Murakami Tomoaki, Inoshima Yasuo, Ishiguro Naotaka

机构信息

Department of Veterinary Medicine, Tokyo University of Agriculture and Technology, Tokyo, Japan.

Department of Veterinary Medicine, Gifu University, Gifu, Japan.

出版信息

Virus Res. 2015 Sep 2;207:76-81. doi: 10.1016/j.virusres.2014.12.019. Epub 2014 Dec 19.

DOI:10.1016/j.virusres.2014.12.019
PMID:25533533
Abstract

Amyloidosis is a collective term for a group of disorders that induce functional impairment of organs and occurs through the accumulation of amyloid, or misfolded protein in beta-sheets. AA amyloidosis is a lethal systemic amyloidosis with SAA as the precursor protein, and is observed in various animal species, including humans. AA amyloidosis can be induced artificially by continuously administering inflammatory stimuli in experimental animal models. In this process of experimental induction, the administration of AA amyloids from either the same or different species is known to markedly expedite AA amyloidosis development, and this is also termed transmission of AA amyloidosis. Similarly to prion disease, AA amyloidosis is considered to be transmitted via a "seeding-nucleation" process. In this manuscript, we reviewed the pathology and transmissibility of AA amyloidosis in animals.

摘要

淀粉样变性是一组导致器官功能障碍的疾病的统称,其通过淀粉样蛋白(即β折叠中的错误折叠蛋白)的积累而发生。AA型淀粉样变性是以血清淀粉样蛋白A(SAA)作为前体蛋白的致死性全身性淀粉样变性,在包括人类在内的各种动物物种中均有观察到。在实验动物模型中,通过持续给予炎症刺激可人工诱导AA型淀粉样变性。在这种实验诱导过程中,已知给予来自相同或不同物种的AA淀粉样蛋白可显著加速AA型淀粉样变性的发展,这也被称为AA型淀粉样变性的传播。与朊病毒病类似,AA型淀粉样变性被认为是通过“种子成核”过程传播的。在本手稿中,我们综述了动物AA型淀粉样变性的病理学和传染性。

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