摘要
从朊病毒蛋白(PrP)的发现到今天已经过去三十多年了,它是成为传染性海绵状脑病的致病原因。自从它获得和失去功能机制合并可能导致传染性海绵状脑病以来,了解朊病毒蛋白生理角色可能在疾病机制方面提供了重要线索。历史上,朊病毒研究的主要策略涉及人类组织、细胞环境和哺乳动物模型的运用。然而,体内研究和有争议的言论在这些系统中存在着实验性困难,这刺激了可替代性动物模型的研究。PrPC在哺乳动物内高度保留的,PrPC相关同源在斑马鱼中表达,脊椎模式生物适用于研究与人类疾病相关机制。脊椎模型,正如它没有表达PrPC,使他们更适应哺乳动物朊病毒蛋白神经毒性机制的调查。在这里,我们回顾在朊病毒蛋白在普通和病理环境下近期研究进展,它基于非哺乳动物研究,强调了斑马鱼,苍蝇和蠕虫对我们理解当代PrP生物所做出的贡献。
关键词: 朊病毒相关疾病、PrPC、生理、神经毒性、哺乳动物、斑马鱼、Drosophila melanogaster、秀丽隐杆线虫。
Current Molecular Medicine
Title:Prion Function and Pathophysiology in Non-Mammalian Models
Volume: 17 Issue: 1
关键词: 朊病毒相关疾病、PrPC、生理、神经毒性、哺乳动物、斑马鱼、Drosophila melanogaster、秀丽隐杆线虫。
摘要: More than thirty years have passed since the discovery of the prion protein (PrP) and its causative role in transmissible spongiform encephalopathy. Since a combination of both gain- and loss-of-function mechanisms may underlay prion pathogenesis, understanding the physiological role of PrP may give important clues about disease mechanisms. Historically, the primary strategy for prion research has involved the use of human tissue, cell cultures and mammalian animal models. Nevertheless, experimental difficulties of in vivo studies and controversial observations obtained in these systems have stimulated the search for alternative animal models. PrPC is highly conserved in mammals, and PrPC-related orthologs are expressed in zebrafish, a vertebrate model organism suitable to study the mechanisms associated with human diseases. Invertebrate models, as they do not express PrPC have served to investigate the neurotoxic mechanisms of mammalian PrP. Here we overview most recent advances in the study of PrP function in normal and pathogenic conditions based on non-mammalian studies, highlighting the contribution of zebrafish, fly and worms to our current understanding of PrP biology.
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Cite this article as:
Prion Function and Pathophysiology in Non-Mammalian Models, Current Molecular Medicine 2017; 17 (1) . https://dx.doi.org/10.2174/1566524017666170220100715
DOI https://dx.doi.org/10.2174/1566524017666170220100715 |
Print ISSN 1566-5240 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5666 |
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