Wadsworth Jonathan D F, Collinge John
MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK.
Biochim Biophys Acta. 2007 Jun;1772(6):598-609. doi: 10.1016/j.bbadis.2007.02.010. Epub 2007 Mar 1.
The recognition that variant Creutzfeldt-Jakob disease (vCJD) is caused by the same prion strain as bovine spongiform encephalopathy in cattle has dramatically highlighted the need for a precise understanding of the molecular biology of human prion diseases. Detailed clinical, pathological and molecular data from a large number of human prion disease patients indicate that phenotypic diversity in human prion disease relates in part to the propagation of disease-related PrP isoforms with distinct physicochemical properties. Incubation periods of prion infection in humans can exceed 50 years and therefore it will be some years before the extent of any human vCJD epidemic can be predicted with confidence.
变异型克雅氏病（vCJD）与牛海绵状脑病由同一朊病毒株引起，这一认识显著凸显了精确理解人类朊病毒病分子生物学的必要性。来自大量人类朊病毒病患者的详细临床、病理和分子数据表明，人类朊病毒病的表型多样性部分与具有不同物理化学性质的疾病相关PrP异构体的传播有关。人类朊病毒感染的潜伏期可能超过50年，因此在能够自信地预测任何人类vCJD疫情的规模之前还需要数年时间。
