Wisconsin National Primate Research Center, University of Wisconsin-Madison, Madison, Wisconsin, United States of America.
Cellular and Molecular Pathology Graduate Program, University of Wisconsin-Madison, Madison, Wisconsin, United States of America.
PLoS One. 2022 Jun 10;17(6):e0269190. doi: 10.1371/journal.pone.0269190. eCollection 2022.
α-Synuclein (α-syn) proteinopathy in the neurons of the Enteric Nervous System (ENS) is proposed to have a critical role in Parkinson's disease (PD) onset and progression. Interestingly, the ENS of the human appendix harbors abundant α-syn and appendectomy has been linked to a decreased risk and delayed onset of PD, suggesting that the appendix may influence PD pathology. Common marmosets and rhesus macaques lack a distinct appendix (a narrow closed-end appendage with a distinct change in diameter at the junction with the cecum), yet the cecal microanatomy of these monkeys is similar to the human appendix. Sections of human appendix (n = 3) and ceca from common marmosets (n = 4) and rhesus macaques (n = 3) were evaluated to shed light on the microanatomy and the expression of PD-related proteins. Analysis confirmed that the human appendix and marmoset and rhesus ceca present thick walls comprised of serosa, muscularis externa, submucosa, and mucosa plus abundant lymphoid tissue. Across all three species, the myenteric plexus of the ENS was located within the muscularis externa with nerve fibers innervating all layers of the appendix/ceca. Expression of α-syn and tau in the appendix/cecum was present within myenteric ganglia and along nerve fibers of the muscularis externa and mucosa in all species. In the myenteric ganglia α-syn, p-α-syn, tau and p-tau immunoreactivities (ir) were not significantly different across species. The percent area above threshold of α-syn-ir and tau-ir in the nerve fibers of the muscularis externa and mucosa were greater in the human appendix than in the NHP ceca (α-syn-ir p<0.05; tau-ir p<0.05). Overall, this study provides critical translational evidence that the common marmoset and rhesus macaque ceca are remarkably similar to the human appendix and, thus, that these NHP species are suitable for studying the development of PD linked to α-syn and tau pathological changes in the ENS.
在肠道神经系统(ENS)的神经元中,α-突触核蛋白(α-syn)蛋白病被认为在帕金森病(PD)的发病和进展中起关键作用。有趣的是,人类阑尾的 ENS 含有丰富的α-syn,阑尾切除术与 PD 风险降低和发病时间延迟有关,这表明阑尾可能影响 PD 病理。普通狨猴和恒河猴没有明显的阑尾(阑尾是一种狭窄的封闭末端附属物,在与盲肠交界处直径有明显变化),但这些猴子的盲肠微解剖结构与人类阑尾相似。评估了人类阑尾(n=3)和普通狨猴(n=4)和恒河猴(n=3)的盲肠切片,以阐明微解剖结构和与 PD 相关的蛋白质表达。分析证实,人类阑尾和狨猴和恒河猴的盲肠均具有由浆膜、外肌层、黏膜下层和黏膜组成的厚壁,并有丰富的淋巴组织。在所有三个物种中,ENS 的肌间神经丛位于外肌层内,神经纤维支配阑尾/盲肠的所有层。在所有三个物种中,α-syn 和 tau 在阑尾/盲肠中的表达均存在于肌间神经节内以及外肌层和黏膜中的神经纤维上。在肌间神经节中,α-syn、p-α-syn、tau 和 p-tau 免疫反应性(ir)在物种间无显著差异。外肌层和黏膜中神经纤维的α-syn-ir 和 tau-ir 阈值以上面积百分比在人类阑尾中高于 NHPs 盲肠(α-syn-ir p<0.05;tau-ir p<0.05)。总的来说,这项研究提供了关键的转化证据,表明普通狨猴和恒河猴的盲肠与人类阑尾非常相似,因此这些 NHPs 物种适合研究与 ENS 中α-syn 和 tau 病理变化相关的 PD 的发展。