Biology Department, William Jewell College, Liberty, MO, 64068, USA.
Department of Biological Sciences, Columbia University, New York, NY, USA.
Neurochem Res. 2018 Dec;43(12):2288-2303. doi: 10.1007/s11064-018-2649-x. Epub 2018 Sep 27.
Gut motility malfunction and pathological changes in the enteric nervous system (ENS) are observed in the early stages of Parkinson's disease (PD). In many cases disturbances in the autonomous functions such as gut motility precedes the observed loss of central motor functions in PD. However, the mechanism by which ENS degeneration occurs in PD is unknown. We show that parkinsonian mimetics rotenone and MPP+ induce neurite degeneration that precedes cell death in primary enteric neurons cultured in vitro. If the neuronal death signals originate from degenerating neurites, neuronal death should be prevented by inhibiting neurite degeneration. Our data demonstrate that overexpression of cytNmnat1, an axon protector, maintains healthy neurites in enteric neurons treated with either of the parkinsonian mimetics, but cannot protect the soma. We also demonstrate that neurite protection via cytNmnat1 is independent of mitochondrial dynamics or ATP levels. Overexpression of Bcl-xl, an anti-apoptotic factor, protects both the neuronal cell body and the neurites in both rotenone and MPP+ treated enteric neurons. Our data reveals that Bcl-xl and cytNmnat1 act through separate mechanisms to protect enteric neurites. Our findings suggest that neurite protection alone is not sufficient to inhibit enteric neuronal degeneration in rotenone or MPP+ toxicity, and enteric neurodegeneration in PD may be occurring through independent somatic and neuritic mechanisms. Thus, therapies targeting both axonal and somal protection can be important in finding interventions for enteric symptoms in PD.
肠道运动功能障碍和肠神经系统(ENS)的病理变化在帕金森病(PD)的早期阶段就可以观察到。在许多情况下,肠道运动等自主功能的紊乱先于 PD 中观察到的中枢运动功能丧失。然而,PD 中 ENS 退化的机制尚不清楚。我们发现帕金森病模拟物鱼藤酮和 MPP+在体外培养的原代肠神经元中诱导神经突退化,随后发生细胞死亡。如果神经元死亡信号源自退化的神经突,那么抑制神经突退化应该可以防止神经元死亡。我们的数据表明,轴突保护蛋白 cytNmnat1 的过表达可以维持帕金森病模拟物处理的肠神经元中健康的神经突,但不能保护神经元胞体。我们还证明,通过 cytNmnat1 进行的神经突保护与线粒体动力学或 ATP 水平无关。抗凋亡因子 Bcl-xl 的过表达可以保护鱼藤酮和 MPP+处理的肠神经元中的神经元胞体和神经突。我们的数据表明,Bcl-xl 和 cytNmnat1 通过独立的机制来保护肠神经突。我们的研究结果表明,神经突保护本身不足以抑制鱼藤酮或 MPP+毒性中的肠神经元退化,PD 中的肠神经退行性变可能通过独立的体和神经突机制发生。因此,针对轴突和体保护的治疗方法可能对寻找 PD 中肠症状的干预措施很重要。