Department of Pathology, National Prion Disease Pathology Surveillance Center, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.
J Mol Neurosci. 2011 Nov;45(3):422-4. doi: 10.1007/s12031-011-9543-1. Epub 2011 May 17.
Variably protease-sensitive prionopathy (VPSPr) is a novel disease involving the prion protein (PrP) that has clinical similarities with non-Alzheimer's dementias especially frontotemporal dementia, diffuse Lewis body disease, and normal pressure hydrocephalus. VPSPr can be distinguished from sporadic Creutzfeldt-Jakob disease (sCJD) especially for the characteristics of the abnormal PrP. Furthermore, although VPSPr like sCJD affects patients with the three PrP genotypes as determined by the common methionine/valine polymorphism, the allelic prevalence is very different in the two diseases. These findings suggest that VPSPr is basically different from classical prion diseases such as sCJD being perhaps more akin to other neurodegenerative dementias.
变异性朊病毒病（VPSPr）是一种新型疾病，涉及朊病毒蛋白（PrP），其临床表现与非阿尔茨海默病性痴呆，尤其是额颞叶痴呆、弥漫性路易体病和正常压力脑积水具有相似性。VPSPr 可与散发性克雅氏病（sCJD）相鉴别，尤其是异常 PrP 的特征不同。此外，尽管 VPSPr 与 sCJD 一样影响由常见蛋氨酸/缬氨酸多态性决定的三种 PrP 基因型的患者，但这两种疾病的等位基因流行率差异很大。这些发现表明，VPSPr 与经典朊病毒病（如 sCJD）基本不同，可能更类似于其他神经退行性痴呆。
