The Roslin Institute TSE Resource Centre

传染性海绵状脑病（Transmissible spongiform encephalopathy，TSE）又称朊病毒病（Prion diseases），是一种危害人类和动物的致死性神经退行性疾病。这些疾病包括牛的牛海绵状脑病（BSE）、绵羊的瘙痒病和人类的克雅氏病（CJD）。TSE是一种传染病，可以在物种内和物种之间传播。据认为，疯牛病通过受污染的食品传播给人类，导致变异型克雅氏病（vCJD）的出现，迄今为止，这种疾病在英国已造成164人死亡。其中4例是在输血后发生的，输血者后来发展为vCJD，这表明vCJD可能发生人传人，并且存在通过手术、牙科和其他侵入性手术进一步人传人的可能性。虽然疯牛病和vCJD的发病率似乎在下降，但不断发现新的动物TSE，如北美鹿和麋鹿中的非典型瘙痒病、非典型形式的疯牛病（H型和L型）和慢性消耗性疾病（CWD）。这些传染性病原体对人类构成的风险尚不清楚。出于这些原因，继续研究疾病的原因和神经变性的机制是必不可少的。只有通过确定TSE感染因子的性质及其如何破坏神经元，才能开发出有效的治疗方法和死亡前诊断方法。为了进行这种研究，需要专门的试剂。TSE病原体菌株只能在动物中真正繁殖，并且此类试剂只能在具有专门设施的实验室中生成或处理。在英国，三个资源中心为TSE研究提供专门的材料; NIBSC的CJD资源中心拥有人类病例的组织，VLA的TSE档案馆拥有大量的绵羊和牛TSE感染组织档案，罗斯林研究所的TSE资源中心提供实验小鼠TSE试剂，抗体和细胞系。从所有三个TSE档案材料的提供是重要的，以维持TSE研究在英国。在向BBR基金提出的这一建议中，我们正在寻求对罗斯林研究所TSE资源中心的支持，这将使其发展成为一个自我维持的资源，并改善目录和在线用户访问。这一发展将保障TSE资源中心的未来，并允许继续向TSE研究界提供由罗斯林研究所神经发病部的研究人员进行的世界级TSE研究所生产的高质量试剂。

The Transmissible spongiform encephalopathy (TSE) diseases (also known as Prion diseases) are fatal neurodegenerative diseases of animals and humans. These include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and Creutzfeldt-Jakob disease (CJD) in humans. TSEs are infectious diseases and can be transmitted within and between species. It is thought that the transmission of BSE to humans via contaminated foodstuffs resulted in the emergence of variant CJD (vCJD), which to date has caused 164 deaths in the UK. Four of these cases have occurred following a blood transfusion from an individual who later went on to develop vCJD, indicating that human to human transmission of vCJD can occur, and the potential exists for further human to human spread via surgery, dentistry and other invasive procedures. Although the incidence of BSE and vCJD appear to be declining, new TSEs of animals are constantly being identified, such as atypical scrapie, atypical forms of BSE (H-type and L-type), and chronic wasting disease (CWD) in North American deer and elk. The risks posed to humans from these infectious agents are unknown. For these reasons, continued research into the causes of disease and mechanisms of neurodegeneration are essential. Only by identifying the nature of the TSE infectious agent and how it destroys neurons will effective therapies and pre-mortem diagnostic assays be developed. In order to perform such research, specialised reagents are required. TSE agent strains can only be truly propagated in animals, and such reagents can only be generated or processed in laboratories with specialised facilities. In the UK, three resource centres supply specialised materials for TSE research; the CJD Resource Centre at NIBSC holds tissue from human cases of disease, the TSE Archive at VLA holds an extensive archive of ovine and bovine TSE infected tissues, and the TSE Resource Centre at the Roslin Institute supplies experimental mouse TSE agents, antibodies and cell lines. The provision of material from all three TSE archives is important to sustain TSE research in the UK. In this proposal to The BBR Fund, we are seeking support for the Roslin Institute TSE Resource Centre that will allow its development into a self sustaining resource with improved catalogue and on-line user access. This development will safeguard the future of the TSE Resource Centre and allow the continued supply of high quality reagents produced as a result of the world class TSE research performed by researchers in the Roslin Institute-Neuropathogenesis Division, to the TSE research community.