Keizo Asami Institute, iLIKA, Universidade Federal de Pernambuco, Moraes Rego Avenue, 1235, Recife, Pernambuco, 50670-901, Brazil.
Department of Pharmacology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
Neurotox Res. 2024 Feb 6;42(1):11. doi: 10.1007/s12640-024-00686-3.
Parkinson's disease (PD) is the second most common neurodegenerative disease in the world, the first being Alzheimer's disease. Patients with PD have a loss of dopaminergic neurons in the substantia nigra of the basal ganglia, which controls voluntary movements, causing a motor impairment as a result of dopaminergic signaling impairment. Studies have shown that mutations in several genes, such as SNCA, PARK2, PINK1, DJ-1, ATP13A2, and LRRK2, and the exposure to neurotoxic agents can potentially increase the chances of PD development. The nematode Caenorhabditis elegans (C. elegans) plays an important role in studying the risk factors, such as genetic factors, aging, exposure to chemicals, disease progression, and drug treatments for PD. C. elegans has a conserved neurotransmission system during evolution; it produces dopamine, through the eight dopaminergic neurons; it can be used to study the effect of neurotoxins and also has strains that express human α-synuclein. Furthermore, the human PD-related genes, LRK-1, PINK-1, PDR-1, DJR-1.1, and CATP-6, are present and functional in this model. Therefore, this review focuses on highlighting and discussing the use of C. elegans an in vivo model in PD-related studies. Here, we identified that nematodes exposed to the neurotoxins, such as 6-OHDA, MPTP, paraquat, and rotenone, had a progressive loss of dopaminergic neurons, dopamine deficits, and decreased survival rate. Several studies have reported that expression of human LRRK2 (G2019S) caused neurodegeneration and pink-1, pdr-1, and djr-1.1 deletion caused several effects PD-related in C. elegans, including mitochondrial dysfunctions. Of note, the deletion of catp-6 in nematodes caused behavioral dysfunction, mitochondrial damage, and reduced survival. In addition, nematodes expressing α-synuclein had neurodegeneration and dopamine-dependent deficits. Therefore, C. elegans can be considered an accurate animal model of PD that can be used to elucidate to assess the underlying mechanisms implicated in PD to find novel therapeutic targets.
帕金森病(PD)是世界上第二常见的神经退行性疾病,第一是阿尔茨海默病。PD 患者的基底神经节黑质中的多巴胺能神经元丧失,这控制着自主运动,导致多巴胺能信号传递受损的运动障碍。研究表明,几个基因的突变,如 SNCA、PARK2、PINK1、DJ-1、ATP13A2 和 LRRK2,以及接触神经毒素,可能会增加 PD 发展的机会。线虫秀丽隐杆线虫(C. elegans)在研究遗传因素、衰老、接触化学物质、疾病进展和 PD 药物治疗等风险因素方面发挥着重要作用。C. elegans 在进化过程中具有保守的神经传递系统;它通过八个多巴胺能神经元产生多巴胺;它可用于研究神经毒素的影响,并且还有表达人类α-突触核蛋白的品系。此外,人类 PD 相关基因 LRK-1、PINK-1、PDR-1、DJR-1.1 和 CATP-6 存在于该模型中并具有功能。因此,本综述重点强调并讨论了使用秀丽隐杆线虫作为 PD 相关研究的体内模型。在这里,我们发现暴露于神经毒素(如 6-OHDA、MPTP、百草枯和鱼藤酮)的线虫多巴胺能神经元逐渐丧失、多巴胺缺乏和存活率降低。几项研究报告称,人类 LRRK2(G2019S)的表达导致神经退行性变,pink-1、pdr-1 和 djr-1.1 的缺失导致线虫中的几种 PD 相关效应,包括线粒体功能障碍。值得注意的是,线虫中 catp-6 的缺失导致行为功能障碍、线粒体损伤和存活率降低。此外,表达α-突触核蛋白的线虫发生神经退行性变和多巴胺依赖性缺陷。因此,秀丽隐杆线虫可以被认为是一种准确的 PD 动物模型,可用于阐明评估 PD 中涉及的潜在机制,以找到新的治疗靶点。