Abstract：
Prion diseases are caused by prions, which are proteinaceous infectious particles that have been identified as causative factors of transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease (CJD). Prion diseases are devastating neurodegenerative disorders in humans and many animals, including sheep, cows, deer, cats, and camels. Prion diseases are classified into sporadic and genetic forms. Additionally, a third, environmentally acquired category exists. This type includes kuru, iatrogenic CJD caused by human dura mater grafts or human pituitary-derived hormones, and variant CJD transmitted through food contaminated with bovine spongiform encephalopathy prions. Bovine spongiform encephalopathy and variant CJD have nearly been controlled, but chronic wasting disease, a prion disease affecting deer, is spreading widely in North America and South Korea and recently in Northern Europe. Recently, amyloid-beta, alpha-synuclein, and other proteins related to Alzheimer\'s disease, Parkinson\'s disease, and other neurodegenerative diseases were reported to have prion features such as transmission to animals. Amyloid-beta transmission to humans has been suggested in iatrogenic CJD cases and in cerebral amyloid angiopathy cases with cerebral bleeding occurring long after childhood neurosurgery with or without cadaveric dura mater transplantation. These findings indicate that diseases caused by various prions, namely various transmissible proteins, appear to be a threat, particularly in the current longevity society. Prion disease represented by CJD has obvious transmissibility and is considered to be an \"archetype of various neurodegenerative diseases\". Overcoming prion diseases is a top priority currently in our society, and this strategy will certainly contribute to elucidating pathomechanism of other neurodegenerative diseases and developing new therapies for them.
摘要：
朊病毒疾病是由朊病毒引起的,它们是蛋白质感染性颗粒，已被确定为传染性海绵状脑病的致病因素，例如克雅氏病（CJD）。朊病毒疾病是人类和许多动物的毁灭性神经退行性疾病，包括羊,奶牛,鹿,猫,还有骆驼.朊病毒疾病分为散发性和遗传形式。此外,第三个,环境获得类别存在。这种类型包括库鲁，由人类硬脑膜移植物或人类垂体源激素引起的医源性CJD，和变异的CJD通过被牛海绵状脑病朊病毒污染的食物传播。牛海绵状脑病和变异型CJD已几乎得到控制，但是慢性消耗性疾病，一种影响鹿的朊病毒病，在北美和韩国以及最近在北欧广泛传播。最近,淀粉样β,α-突触核蛋白,和其他与阿尔茨海默病有关的蛋白质，帕金森病,据报道，其他神经退行性疾病具有朊病毒特征，例如传播给动物。在医源性CJD病例和脑淀粉样血管病病例中，有或没有尸体硬脑膜移植的儿童期神经外科手术后很久就发生了脑出血，已经提出了淀粉样蛋白β向人类的传播。这些发现表明，由各种朊病毒引起的疾病，即各种可传播的蛋白质，似乎是一种威胁,特别是在当前的长寿社会。以CJD为代表的朊病毒病具有明显的传播性，被认为是“各种神经退行性疾病的原型”。克服朊病毒疾病是我们社会目前的首要任务，这种策略肯定会有助于阐明其他神经退行性疾病的病理机制并为其开发新疗法。
