Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy.
Prog Mol Biol Transl Sci. 2017;150:409-431. doi: 10.1016/bs.pmbts.2017.05.004. Epub 2017 Jul 3.
Prion diseases are unique neurodegenerative pathologies that can occur with sporadic, genetic, and acquired etiologies. Human and animal prion diseases can be recapitulated in laboratory animals with good reproducibility providing highly controlled models for studying molecular mechanisms of neurodegeneration. In this chapter the overall area of omics research in prion diseases is described. The term omics includes all fields of studies that employ a comprehensive, unbiased, and high-throughput approach to areas of research such as functional genomics, transcriptomics, and proteomics. These kind of approaches can be extremely helpful in identifying disease susceptibility factors and pathways that are dysregulated upon the onset and the progression of the disease. Herein, the most important research about the various forms of prion pathologies in human and in models of prion diseases in animals is presented and discussed.
朊病毒病是一种独特的神经退行性疾病，可由散发性、遗传性和获得性病因引起。人类和动物朊病毒病可以在实验室动物中重现，具有良好的重现性，为研究神经退行性变的分子机制提供了高度可控的模型。本章描述了朊病毒病的组学研究的总体领域。组学一词包括所有采用全面、无偏和高通量方法研究功能基因组学、转录组学和蛋白质组学等领域的研究。这些方法在识别疾病易感性因素和疾病发生和进展时失调的途径方面非常有帮助。本文介绍并讨论了朊病毒病在人类和动物朊病毒病模型中的各种形式的朊病毒病的最重要的研究。
