Prion disease: experimental models and reality

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• 作者：Sebastian Brandner ; Zane Jaunmuktane
• 刊名：Acta Neuropathologica
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：133
• 期：2
• 页码：197-222
• 全文大小：1425KB
• 刊物类别：Medicine
• 刊物主题：Pathology; Neurosciences;
• 出版者：Springer Berlin Heidelberg
• ISSN：1432-0533
• 卷排序：133

文摘

The understanding of the pathogenesis and mechanisms of diseases requires a multidisciplinary approach, involving clinical observation, correlation to pathological processes, and modelling of disease mechanisms. It is an inherent challenge, and arguably impossible to generate model systems that can faithfully recapitulate all aspects of human disease. It is, therefore, important to be aware of the potentials and also the limitations of specific model systems. Model systems are usually designed to recapitulate only specific aspects of the disease, such as a pathological phenotype, a pathomechanism, or to test a hypothesis. Here, we evaluate and discuss model systems that were generated to understand clinical, pathological, genetic, biochemical, and epidemiological aspects of prion diseases. Whilst clinical research and studies on human tissue are an essential component of prion research, much of the understanding of the mechanisms governing transmission, replication, and toxicity comes from in vitro and in vivo studies. As with other neurodegenerative diseases caused by protein misfolding, the pathogenesis of prion disease is complex, full of conundra and contradictions. We will give here a historical overview of the use of models of prion disease, how they have evolved alongside the scientific questions, and how advancements in technologies have pushed the boundaries of our understanding of prion biology.