Division of Pharmacology, CSIR-Central Drug Research Institute, Lucknow, U.P., India.
Division of Pharmacology, CSIR-Central Drug Research Institute, Lucknow, U.P., India; Academy of Scientific and Innovative Research, New Delhi, India.
Free Radic Biol Med. 2018 Dec;129:73-87. doi: 10.1016/j.freeradbiomed.2018.08.033. Epub 2018 Aug 31.
Wnts and the components of Wnt/β-catenin signaling are widely expressed in midbrain and required to control the fate specification of dopaminergic (DAergic) neurons, a neuronal population that specifically degenerate in Parkinson's disease (PD). Accumulating evidence suggest that mitochondrial dysfunction plays a key role in pathogenesis of PD. Axin-2, a negative regulator of Wnt/β-catenin signaling affects mitochondrial biogenesis and death/birth of new DAergic neurons is not fully explored. We investigated the functional role of Axin-2/Wnt/β-catenin signaling in mitochondrial biogenesis and DAergic neurogenesis in 6-hydroxydopamine (6-OHDA) induced rat model of PD-like phenotypes. We demonstrate that single unilateral injection of 6-OHDA into the medial forebrain bundle (MFB) potentially dysregulates Wnt/β-catenin signaling in substantia nigra pars compacta (SNpc). We used shRNA lentiviruses to genetically knockdown Axin-2 to up-regulate Wnt/β-catenin signaling in SNpc in parkinsonian rats. Genetic knockdown of Axin-2 up-regulates Wnt/β-catenin signaling by destabilizing the β-catenin degradation complex in SNpc in parkinsonian rats. Axin-2 shRNA mediated activation of Wnt/β-catenin signaling improved behavioural functions and protected the nigral DAergic neurons by increasing mitochondrial functionality in parkinsonian rats. Axin-2 shRNA treatment reduced apoptotic signaling, autophagy and ROS generation and improved mitochondrial membrane potential which promotes mitochondrial biogenesis in SNpc in parkinsonian rats. Interestingly, Axin-2 shRNA-mediated up-regulation of Wnt/β-catenin signaling enhanced net DAergic neurogenesis by regulating proneural genes (Nurr-1, Pitx-3, Ngn-2, and NeuroD1) and mitochondrial biogenesis in SNpc in parkinsonian rats. Therefore, our data suggest that pharmacological/genetic manipulation of Wnt signaling that enhances the endogenous regenerative capacity of DAergic neurons may have implication for regenerative approaches in PD.
Wnt 及其β-连环蛋白信号成分广泛表达于中脑,并控制多巴胺能(DAergic)神经元的命运特化,而多巴胺能神经元在帕金森病(PD)中特异性退化。越来越多的证据表明,线粒体功能障碍在 PD 的发病机制中起着关键作用。轴蛋白-2(Axin-2)是 Wnt/β-连环蛋白信号的负调节剂,影响线粒体生物发生,而新的 DAergic 神经元的生与死尚未完全被探索。我们研究了 Axin-2/Wnt/β-连环蛋白信号在帕金森病样表型的 6-羟多巴胺(6-OHDA)诱导的大鼠模型中线粒体生物发生和 DAergic 神经发生中的功能作用。我们证明,单侧内侧前脑束(MFB)注射 6-OHDA 可能会使黑质致密部(SNpc)中的 Wnt/β-连环蛋白信号失调。我们使用 shRNA 慢病毒来遗传敲低 SNpc 中的 Axin-2,以在上帕金森大鼠中上调 Wnt/β-连环蛋白信号。遗传敲低 Axin-2 通过破坏帕金森大鼠 SNpc 中的β-连环蛋白降解复合物而上调 Wnt/β-连环蛋白信号。Axin-2 shRNA 介导的 Wnt/β-连环蛋白信号激活改善了行为功能,并通过增加帕金森大鼠中 SNpc 的线粒体功能保护黑质 DAergic 神经元。Axin-2 shRNA 治疗减少了凋亡信号、自噬和 ROS 生成,并改善了线粒体膜电位,从而促进了帕金森大鼠 SNpc 中的线粒体生物发生。有趣的是,Axin-2 shRNA 介导的 Wnt/β-连环蛋白信号上调通过调节前体细胞基因(Nurr-1、Pitx-3、Ngn-2 和 NeuroD1)和帕金森大鼠 SNpc 中的线粒体生物发生来增强净 DAergic 神经发生。因此,我们的数据表明,Wnt 信号的药理学/遗传操纵增强了 DAergic 神经元的内源性再生能力,这可能对 PD 的再生方法具有意义。