Fazzari Maria, Lunghi Giulia, Henriques Alexandre, Callizot Noëlle, Ciampa Maria Grazia, Mauri Laura, Prioni Simona, Carsana Emma Veronica, Loberto Nicoletta, Aureli Massimo, Mari Luigi, Sonnino Sandro, Chiricozzi Elena, Di Biase Erika
Department of Medical Biotechnology and Translational Medicine, University of Milano, 20054 Segrate, MI, Italy.
Neuro-Sys, 410 Chemin Départemental 60, 13120 Gardanne, France.
Biomedicines. 2023 Apr 28;11(5):1305. doi: 10.3390/biomedicines11051305.
Past evidence has shown that the exogenous administration of GM1 ganglioside slowed neuronal death in preclinical models of Parkinson's disease, a neurodegenerative disorder characterized by the progressive loss of dopamine-producing neurons: however, the physical and chemical properties of GM1 (i.e., amphiphilicity) limited its clinical application, as the crossing of the blood-brain barrier is denied. Recently, we demonstrated that the GM1 oligosaccharide head group (GM1-OS) is the GM1 bioactive portion that, interacting with the TrkA-NGF complex at the membrane surface, promotes the activation of a multivariate network of intracellular events regulating neuronal differentiation, protection, and reparation. Here, we evaluated the GM1-OS neuroprotective potential against the Parkinson's disease-linked neurotoxin MPTP, which destroys dopaminergic neurons by affecting mitochondrial bioenergetics and causing ROS overproduction. In dopaminergic and glutamatergic primary cultures, GM1-OS administration significantly increased neuronal survival, preserved neurite network, and reduced mitochondrial ROS production enhancing the mTOR/Akt/GSK3β pathway. These data highlight the neuroprotective efficacy of GM1-OS in parkinsonian models through the implementation of mitochondrial function and reduction in oxidative stress.
过去的证据表明,在帕金森病的临床前模型中外源性给予神经节苷脂GM1可减缓神经元死亡,帕金森病是一种以产生多巴胺的神经元进行性丧失为特征的神经退行性疾病:然而,GM1的物理和化学性质(即两亲性)限制了其临床应用,因为它无法穿过血脑屏障。最近,我们证明GM1寡糖头部基团(GM1-OS)是GM1的生物活性部分,它在膜表面与TrkA-NGF复合物相互作用,促进调节神经元分化、保护和修复的细胞内事件多元网络的激活。在这里,我们评估了GM1-OS对与帕金森病相关的神经毒素MPTP的神经保护潜力,MPTP通过影响线粒体生物能量学和导致活性氧(ROS)过量产生来破坏多巴胺能神经元。在多巴胺能和谷氨酸能原代培养物中,给予GM1-OS可显著提高神经元存活率、保留神经突网络并减少线粒体ROS产生,增强mTOR/Akt/GSK3β信号通路。这些数据突出了GM1-OS通过改善线粒体功能和降低氧化应激在帕金森病模型中的神经保护作用。