Jiangsu Key Laboratory of Neurodegeneration, Department of Pharmacology, Nanjing Medical University, Nanjing 211166, China.
Department of Molecular Cell Biology & Toxicology, Center for Global Health, School of Public Health, Nanjing Medical University, 211166, Nanjing, China; Changzhou Second People's Hospital, Changzhou Medical Center, Nanjing Medical University, 213000, Changzhou, China.
Redox Biol. 2024 Jul;73:103190. doi: 10.1016/j.redox.2024.103190. Epub 2024 May 13.
Parkinson's disease (PD) poses a significant challenge in neurodegenerative disorders, characterized by the progressive loss of dopaminergic (DA) neurons in the substantia nigra pars compacta (SNc). The intricate mechanisms orchestrating DA neurodegeneration in PD are not fully understood, necessitating the exploration of innovative therapeutic approaches. Recent studies have implicated ferroptosis as a major contributor to the loss of DA neurons, revealing a complex interplay between iron accumulation and neurodegeneration. However, the sophisticated nature of this process challenges the conventional belief that mere iron removal could effectively prevent DA neuronal ferroptosis. Here, we report JWA, alternatively referred to as ARL6IP5, as a negative regulator of ferroptosis, capable of ameliorating DA neuronal loss in the context of PD. In this study, synchronized expression patterns of JWA and tyrosine hydroxylase (TH) in PD patients and mice were observed, underscoring the importance of JWA for DA neuronal survival. Screening of ferroptosis-related genes unraveled the engagement of iron metabolism in the JWA-dependent inhibition of DA neuronal ferroptosis. Genetic manipulation of JWA provided compelling evidence linking its neuroprotective effects to the attenuation of NCOA4-mediated ferritinophagy. Molecular docking, co-immunoprecipitation, and immunofluorescence studies confirmed that JWA mitigated DA neuronal ferroptosis by occupying the ferritin binding site of NCOA4. Moreover, the JWA-activating compound, JAC4, demonstrated promising neuroprotective effects in cellular and animal PD models by elevating JWA expression, offering a potential avenue for neuroprotection in PD. Collectively, our work establishes JWA as a novel regulator of ferritinophagy, presenting a promising therapeutic target for addressing DA neuronal ferroptosis in PD.
帕金森病(PD)是一种神经退行性疾病,其特征是黑质致密部(SNc)中的多巴胺能(DA)神经元进行性丧失。调控 PD 中 DA 神经元神经退行性变的复杂机制尚未完全阐明,需要探索创新的治疗方法。最近的研究表明铁死亡是导致 DA 神经元丧失的主要因素之一,揭示了铁积累与神经退行性变之间的复杂相互作用。然而,这个过程的复杂性挑战了传统的观念,即仅仅去除铁就可以有效地防止 DA 神经元铁死亡。在这里,我们报告 JWA,也称为 ARL6IP5,作为铁死亡的负调节剂,能够改善 PD 中 DA 神经元的丧失。在这项研究中,观察到 JWA 和酪氨酸羟化酶(TH)在 PD 患者和小鼠中的同步表达模式,强调了 JWA 对 DA 神经元存活的重要性。铁死亡相关基因的筛选揭示了铁代谢在 JWA 依赖的抑制 DA 神经元铁死亡中的参与。JWA 的遗传操作提供了令人信服的证据,将其神经保护作用与 NCOA4 介导的铁蛋白自噬的衰减联系起来。分子对接、共免疫沉淀和免疫荧光研究证实,JWA 通过占据 NCOA4 的铁蛋白结合位点来减轻 DA 神经元铁死亡。此外,JWA 激活化合物 JAC4 通过提高 JWA 表达,在细胞和动物 PD 模型中显示出有希望的神经保护作用,为 PD 中的神经保护提供了一个潜在的途径。总之,我们的工作确立了 JWA 作为铁蛋白自噬的新型调节因子,为解决 PD 中的 DA 神经元铁死亡提供了一个有前途的治疗靶点。