U.S. Based Collaboration on Prion Diseases

慢性消耗性疾病（CWD）是最近在美国西部出现的一种水平传播的鹿和麋鹿传染性海绵状脑病（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”）传播给人类并引起新的TSE（变异型克雅氏病，vCJD）的先例，需要谨慎。该项目的目标是了解慢性消耗病在野外传播的机制、影响其向其他物种传播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CWD朊病毒剂在本地鹿种血液、肌肉、排泄物和唾液中的潜在感染性和传播性。 开发鹿PrPc转基因小鼠，研究慢性消耗病朊病毒感染性、传播、宿主范围、发病机制和治疗方法。 评估CWD的种间传播和发病机制的潜力。 评估PrP肽免疫以预防鹿的CWD感染。 开发动物模型、接种物、设施和检测方法，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、体液和接种物来自所有在接种CWD后发展TSE的物种，并向其他研究者提供CWD特异性试剂和测定方案。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.