CIC bioGUNE, Parque tecnológico de Bizkaia, Derio 48160, Bizkaia, Spain.
Curr Top Med Chem. 2013;13(19):2504-21. doi: 10.2174/15680266113136660177.
Prion diseases belong to a group of fatal infectious diseases with no effective therapies available. Throughout the last 35 years, less than 50 different drugs have been tested in different experimental animal models without hopeful results. An important limitation when searching for new drugs is the existence of appropriate models of the disease. The three different possible origins of prion diseases require the existence of different animal models for testing anti-prion compounds. Wild type, over-expressing transgenic mice and other more sophisticated animal models have been used to evaluate a diversity of compounds which some of them were previously tested in different in vitro experimental models. The complexity of prion diseases will require more pre-screening studies, reliable sporadic (or spontaneous) animal models and accurate chemical modifications of the selected compounds before having an effective therapy against human prion diseases. This review is intended to put on display the more relevant animal models that have been used in the search of new antiprion therapies and describe some possible procedures when handling chemical compounds presumed to have anti-prion activity prior to testing them in animal models.
朊病毒病属于一组致命的传染病，目前尚无有效的治疗方法。在过去的 35 年中，在不同的实验动物模型中测试了不到 50 种不同的药物，但没有取得有希望的结果。在寻找新药时，一个重要的限制因素是存在合适的疾病模型。朊病毒病的三种不同可能起源需要存在不同的动物模型来测试抗朊病毒化合物。野生型、过表达转基因小鼠和其他更复杂的动物模型已被用于评估多种化合物，其中一些化合物之前已在不同的体外实验模型中进行了测试。朊病毒病的复杂性将需要更多的预筛选研究、可靠的散发性（或自发性）动物模型以及对选定化合物进行准确的化学修饰，然后才能对人类朊病毒病进行有效的治疗。本文旨在展示在寻找新的抗朊病毒治疗方法中使用的更相关的动物模型，并描述在将假定具有抗朊病毒活性的化学化合物在动物模型中进行测试之前处理这些化合物的一些可能程序。
