Prion disease modelled in Drosophila.
Prion disease modelled in Drosophila.
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DOI：
10.1007/s00441-022-03586-0
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发表时间：
2023-04
影响因子：
3.6
通讯作者：
中科院分区：
文献类型：
--
作者：
朊病毒疾病是人类和各种脊椎动物的致命神经退行性疾病，可在相同或不同物种的个体之间传播。一种被称为朊病毒的新型传染性片段被认为是传播这些疾病的原因。朊病毒复制被认为是在朊病毒疾病发病过程中产生神经毒性的原因。朊病毒假说预测，可传播的朊病毒病原体由PrPSc组成，PrPSc由正常宿主蛋白PrPC的聚集错误折叠构象组成。了解传染性朊病毒的生物学和鉴定朊病毒诱导的神经毒性的遗传修饰因子是很重要的。这一信息将为制定人类和动物朊病毒疾病的治疗和控制战略提供基础。检测朊病毒传染性最可靠的方法是在合适的实验宿主体内传播，迄今为止，实验宿主一直是哺乳动物。目前的朊病毒生物测定方法缓慢、繁琐，对低滴度的朊病毒传染性相对不敏感，也不适合对朊病毒疾病进行快速遗传分析。在这里，我们概述了我们的新研究，这些研究导致了黑腹果蝇的建立，这是一种遗传上定义良好的无脊椎动物宿主，作为一种敏感、通用和经济上可行的动物模型，用于检测哺乳动物朊病毒传染性和朊病毒诱导毒性的遗传修饰剂。
Prion diseases are fatal neurodegenerative conditions of humans and various vertebrate species that are transmissible between individuals of the same or different species. A novel infectious moiety referred to as a prion is considered responsible for transmission of these conditions. Prion replication is believed to be the cause of the neurotoxicity that arises during prion disease pathogenesis. The prion hypothesis predicts that the transmissible prion agent consists of PrPSc, which is comprised of aggregated misfolded conformers of the normal host protein PrPC. It is important to understand the biology of transmissible prions and to identify genetic modifiers of prion-induced neurotoxicity. This information will underpin the development of therapeutic and control strategies for human and animal prion diseases. The most reliable method to detect prion infectivity is by in vivo transmission in a suitable experimental host, which to date have been mammalian species. Current prion bioassays are slow, cumbersome and relatively insensitive to low titres of prion infectivity, and do not lend themselves to rapid genetic analysis of prion disease. Here, we provide an overview of our novel studies that have led to the establishment of Drosophila melanogaster, a genetically well-defined invertebrate host, as a sensitive, versatile and economically viable animal model for the detection of mammalian prion infectivity and genetic modifiers of prion-induced toxicity.
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