U.S. Based Collaboration on Prion Diseases

慢性消耗性脑病(CWD)是最近在美国西部出现的一种由鹿和麋鹿引起的水平传播性海绵状脑病(TSE)。虽然没有证据表明这种疾病可以传播给人类，但由于牛海绵状脑病(BSE，又名“疯牛病”)传播给人类并导致一种新的TSE(变异型克雅氏病，vCJD)的先例，有必要谨慎行事。该项目的目标是了解慢性萎缩性脑病在野外传播的机制，影响其传播到其他物种的因素，以及检测和控制人类接触受感染动物的方法。其他目标包括： 评估本地鹿种血液、肌肉、排泄物和唾液中CWD病毒的潜在感染性和传播性。 建立CWD PrPc转基因小鼠，研究CWD感染、传播、宿主范围、发病机制和治疗方法。 评估CWD跨物种传播的可能性和发病机制。 评估PrP多肽免疫预防CWD感染的效果。 ？开发动物模型、疫苗、设施和化验，使调查小组能够快速应对与TSE自然出现相关的问题。该项目将开发一个组织、体液和接种物的储存库，这些组织、体液和接种物来自接种CWD后出现TSE的所有物种，并使CWD特有的试剂和检测方案可供其他研究人员使用。组织和试剂将被编目，并提供给在TSE领域工作的其他科学家。

Chronic wasting disease (CWD) is a horizontally transmitted transmissible spongiform encephalopathy (TSE) of deer and elk recently emergent in the Western United States. Although there is no evidence that this disease can be transmitted to humans, caution is warranted due to the precedent of Bovine Spongiform Encephalopathy (BSE, aka "Mad Cow Disease") transmitting to humans and giving rise to a new TSE (variant Creutzfeldt-Jakob disease, vCJD). This project has the objective of understanding the mechanisms by which CWD is transmitted in the wild, the factors that affect its spread to other species, and approaches for detecting, and thus controlling human exposure to, infected animals. Additional objectives include: ¿ Assess the potential infectivity and thus transmissibility of the CWD prion agent in blood, muscle, excreta, and saliva in the native deer species. ¿ Develop cervid PrPc transgenic mice to study CWD prion infectivity, transmission, host range, pathogenesis, and therapeutics. ¿ Evaluate the potential for inter-species transmission and pathogenesis of CWD. ¿ Evaluate a PrP peptide immunization to prevent CWD infection in deer. ¿ Develop animal models, inocula, facilities, and assays that will allow the investigative team to quickly respond to issues relevant to the natural emergence of TSEs. This project will develop a repository of tissues, body fluids, and inocula from all species which develop TSE following CWD inoculation and make CWD-specific reagents and assay protocols available to other investigators. Tissues and reagents will be cataloged and made available to other scientists working in the TSE field.