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代偿性突触结合蛋白-11表达掩盖了帕金蛋白基因敲除小鼠的帕金森病样表型。

Compensatory synaptotagmin-11 expression conceals parkinson's disease-like phenotypes in parkin knockout mice.

作者信息

Dong Nan, Xie Zhenli, Wei Anqi, Yang Yuxin, Deng Yongning, Cheng Xu, Wang Bianbian, Chen Yang, Gu Yuhao, Yao Jingyu, Qin Yuhao, Zheng Chaowen, Zhang Xi, Zhang Yuqing, Kang Xinjiang, Chen Guoqing, Qu Qiumin, Wang Changhe, Xu Huadong

机构信息

Department of Neurology, the Second Affiliated Hospital, Neuroscience Research Center, Key Laboratory of Biomedical Information Engineering of Ministry of Education, School of Life Science and Technology, Xi'an Jiaotong University, Xi'an, 710049, China.

Key Laboratory of Medical Electrophysiology, Collaborative Innovation Center for Prevention and Treatment of Cardiovascular Disease, Institute of Cardiovascular Research, Ministry of Education of China, Southwest Medical University, Luzhou, 646000, China.

出版信息

Cell Commun Signal. 2025 Feb 3;23(1):61. doi: 10.1186/s12964-025-02037-x.

Abstract

Animal models are crucial for elucidating the pathological mechanisms underlying Parkinson's disease (PD). Unfortunately, most of transgenic mouse models fail to manifest pathological changes observed in PD patients, pending the advancement of PD research. However, the mechanism underlying this discrepancy remains elusive. Here, we provide compelling evidence that the compensatory expression of synaptotagmin-11 (Syt11) plays a key role in concealing PD-associated phenotypes in parkin knockout (KO) mouse models. Unlike the normal dopamine (DA) release and motor behaviors observed in parkin KO mice, parkin knockdown (KD) in the substantia nigra pars compacta (SNpc) in adult mice led to both the impaired DA release and the pronounced motor deficits. Interestingly, Syt11, a well-established parkin substrate involved in PD, was specifically upregulated in parkin KD mice and in parkin KO mice during the suckling stage, but not in adult parkin KO mice. Importantly, the overexpression of Syt11 alone is capable of inducing PD-like motor and non-motor impairments, as well as the impaired DA release and reuptake, which is essential for parkin-associated pathogenesis of PD. Therefore, this work not only elucidate a compensatory mechanism that accounts for the absence of overt PD phenotypes in parkin KO mice, but also contribute to the comprehensive understanding of the progression of PD, opening new avenues for the therapeutic treatment of PD.

摘要

动物模型对于阐明帕金森病(PD)的病理机制至关重要。不幸的是,大多数转基因小鼠模型未能表现出PD患者中观察到的病理变化,这使得PD研究进展缓慢。然而,这种差异背后的机制仍然难以捉摸。在这里,我们提供了令人信服的证据,表明突触结合蛋白-11(Syt11)的代偿性表达在掩盖帕金森蛋白基因敲除(KO)小鼠模型中与PD相关的表型方面起着关键作用。与帕金森蛋白基因敲除小鼠中观察到的正常多巴胺(DA)释放和运动行为不同,成年小鼠黑质致密部(SNpc)中帕金森蛋白的敲低(KD)导致DA释放受损和明显的运动缺陷。有趣的是,Syt11是一种与PD相关的、已被充分证实的帕金森蛋白底物,在哺乳期的帕金森蛋白基因敲除小鼠和帕金森蛋白基因敲除小鼠中特异性上调,但在成年帕金森蛋白基因敲除小鼠中则不然。重要的是,单独过表达Syt11能够诱导类似PD的运动和非运动损伤,以及DA释放和再摄取受损,这对于帕金森蛋白相关的PD发病机制至关重要。因此,这项工作不仅阐明了一种解释帕金森蛋白基因敲除小鼠中缺乏明显PD表型的代偿机制,而且有助于全面理解PD的进展,为PD的治疗开辟了新途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e36f/11789404/634b06abc85c/12964_2025_2037_Fig1_HTML.jpg

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