Prion Research Center, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, USA; Department of Microbiology, Immunology and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, USA.
Research Division, Arkansas Game and Fish Commission, Little Rock, Arkansas, USA.
J Biol Chem. 2022 Apr;298(4):101834. doi: 10.1016/j.jbc.2022.101834. Epub 2022 Mar 16.
Chronic wasting disease (CWD) is an invariably fatal prion disease affecting cervid species worldwide. Prions can manifest as distinct strains that can influence disease pathology and transmission. CWD is profoundly lymphotropic, and most infected cervids likely shed peripheral prions replicated in lymphoid organs. However, CWD is a neurodegenerative disease, and most research on prion strains has focused on neurogenic prions. Thus, a knowledge gap exists comparing neurogenic prions to lymphogenic prions. In this study, we compared prions from the obex and lymph nodes of naturally exposed white-tailed deer to identify potential biochemical strain differences. Here, we report biochemical evidence of strain differences between the brain and lymph node from these animals. Conformational stability assays, glycoform ratio analyses, and immunoreactivity scanning across the structured domain of the prion protein that refolds into the amyloid aggregate of the infectious prion reveal significantly more structural and glycoform variation in lymphogenic prions than neurogenic prions. Surprisingly, we observed greater biochemical differences among neurogenic prions than lymphogenic prions across individuals. We propose that the lymphoreticular system propagates a diverse array of prions from which the brain selects a more restricted pool of prions that may be quite different than those from another individual of the same species. Future work should examine the biological and zoonotic impact of these biochemical differences and examine more cervids from multiple locations to determine if these differences are conserved across species and locations.
慢性消耗性疾病(CWD)是一种全球性的、致命的朊病毒疾病,影响鹿科动物。朊病毒可以表现为不同的株,从而影响疾病的病理和传播。CWD 具有明显的淋巴亲嗜性,大多数受感染的鹿科动物可能会在淋巴器官中复制外周朊病毒。然而,CWD 是一种神经退行性疾病,大多数关于朊病毒株的研究都集中在神经起源的朊病毒上。因此,在比较神经起源的朊病毒和淋巴起源的朊病毒方面存在知识差距。在这项研究中,我们比较了自然暴露的白尾鹿的 obex 和淋巴结中的朊病毒,以确定潜在的生化株差异。在这里,我们报告了这些动物的大脑和淋巴结之间的生化株差异的证据。构象稳定性测定、糖型比分析以及对朊病毒蛋白结构域的免疫反应性扫描,该结构域折叠成感染性朊病毒的淀粉样聚集体,揭示了淋巴起源的朊病毒比神经起源的朊病毒具有更多的结构和糖型变异。令人惊讶的是,我们观察到个体之间的神经起源的朊病毒之间的生化差异比淋巴起源的朊病毒更大。我们提出,淋巴网状系统传播了各种各样的朊病毒,大脑从中选择了一个更有限的朊病毒池,这些朊病毒可能与同一物种的另一个个体的朊病毒非常不同。未来的工作应该研究这些生化差异的生物学和人畜共患病影响,并检查来自多个地点的更多的鹿科动物,以确定这些差异是否在物种和地点之间是保守的。