Tranulis Michael A, Tryland Morten
Department of Preclinical Sciences and Pathology, Faculty of Veterinary Medicine, Norwegian University of Life Sciences, 5003 As, Norway.
Department of Forestry and Wildlife Management, Faculty of Applied Ecology, Agricultural Sciences and Biotechnology, Inland Norway University of Applied Sciences, 2480 Koppang, Norway.
Foods. 2023 Feb 15;12(4):824. doi: 10.3390/foods12040824.
Prion diseases are transmissible neurodegenerative disorders that affect humans and ruminant species consumed by humans. Ruminant prion diseases include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep and goats and chronic wasting disease (CWD) in cervids. In 1996, prions causing BSE were identified as the cause of a new prion disease in humans; variant Creutzfeldt-Jakob disease (vCJD). This sparked a food safety crisis and unprecedented protective measures to reduce human exposure to livestock prions. CWD continues to spread in North America, and now affects free-ranging and/or farmed cervids in 30 US states and four Canadian provinces. The recent discovery in Europe of previously unrecognized CWD strains has further heightened concerns about CWD as a food pathogen. The escalating CWD prevalence in enzootic areas and its appearance in a new species (reindeer) and new geographical locations, increase human exposure and the risk of CWD strain adaptation to humans. No cases of human prion disease caused by CWD have been recorded, and most experimental data suggest that the zoonotic risk of CWD is very low. However, the understanding of these diseases is still incomplete (e.g., origin, transmission properties and ecology), suggesting that precautionary measures should be implemented to minimize human exposure.
朊病毒病是一种可传播的神经退行性疾病，会影响人类以及被人类食用的反刍动物物种。反刍动物朊病毒病包括牛的牛海绵状脑病（BSE）、绵羊和山羊的痒病以及鹿科动物的慢性消耗性疾病（CWD）。1996年，导致BSE的朊病毒被确定为人类一种新型朊病毒病——变异型克雅氏病（vCJD）的病因。这引发了一场食品安全危机以及前所未有的保护措施，以减少人类接触家畜朊病毒。CWD继续在北美传播，目前已影响到美国30个州和加拿大4个省的野生和/或养殖鹿科动物。最近在欧洲发现了以前未被识别的CWD毒株，这进一步加剧了人们对CWD作为一种食物病原体的担忧。CWD在疫区的患病率不断上升，并且在一个新物种（驯鹿）和新的地理位置出现，增加了人类接触以及CWD毒株适应人类的风险。目前尚未记录到由CWD引起的人类朊病毒病病例，大多数实验数据表明CWD的人畜共患病风险非常低。然而，对这些疾病的了解仍然不完整（例如，起源、传播特性和生态学），这表明应采取预防措施以尽量减少人类接触。
