Central Laboratory, The Third Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, Jiangsu, China.
Clinical Biobank of Nanjing Hospital of Chinese Medicine, The Third Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, Jiangsu, China.
J Cell Mol Med. 2017 Dec;21(12):3761-3775. doi: 10.1111/jcmm.13285. Epub 2017 Aug 2.
Parkinson's disease (PD) is one of the most common neurodegenerative diseases. Recent epidemiological studies suggest that echinacoside (ECH), a phenylethanoid glycoside found in Cistanche deserticola, has a protective effect against the development of PD. However, the detailed mechanisms of how ECH suppresses neuronal death have not been fully elucidated. In this study, we confirmed that ECH protects nigrostriatal neurons against 6-hydroxydopamine (6-OHDA)-induced endoplasmic reticulum stress (ERS) in vivo and in vitro. ECH rescued cell viability in damaged cells and decreased 6-OHDA-induced reactive oxygen species accumulation in vitro. It also rescued tyrosine hydroxylase and dopamine transporter expression in the striatum, and decreased α-synuclein aggregation following 6-OHDA treatment in vivo. The validated mechanism of ECH activity was the reduction in the 6-OHDA-induced accumulation of seipin (Berardinelli-Seip congenital lipodystrophy 2). Seipin has been shown to be a key molecule related to motor neuron disease and was tightly associated with ERS in a series of in vivo studies. ECH attenuated seipinopathy by promoting seipin degradation via ubiquitination. ERS was relieved by ECH through the Grp94/Bip-ATF4-CHOP signal pathway.
帕金森病(PD)是最常见的神经退行性疾病之一。最近的流行病学研究表明,从管花肉苁蓉中提取的苯乙醇苷类化合物——松果菊苷(ECH)对 PD 的发展具有保护作用。然而,ECH 抑制神经元死亡的详细机制尚未完全阐明。在本研究中,我们证实 ECH 可在体内和体外保护黑质纹状体神经元免受 6-羟多巴胺(6-OHDA)诱导的内质网应激(ERS)。ECH 挽救了受损细胞中的细胞活力,并减少了体外 6-OHDA 诱导的活性氧积累。它还挽救了纹状体中酪氨酸羟化酶和多巴胺转运体的表达,并减少了体内 6-OHDA 处理后α-突触核蛋白的聚集。ECH 活性的验证机制是减少 6-OHDA 诱导的 seipin(先天性脂肪营养不良 2 型)积累。在一系列体内研究中,已经表明 seipin 是与运动神经元疾病相关的关键分子,并且与 ERS 密切相关。ECH 通过促进泛素化降解 seipin 来减轻 seipinopathy。ECH 通过 Grp94/Bip-ATF4-CHOP 信号通路缓解 ERS。