Lee Sang Hoon, Tonello Raquel, Lee Kihwan, Roh Jueun, Prudente Arthur Silveira, Kim Yong Ho, Park Chul-Kyu, Berta Temugin
Pain Research Center, Department of Anesthesiology, University of Cincinnati Medical Center, Cincinnati, OH 45267, USA.
Pain Research Center, Department of Molecular Pathobiology, College of Dentistry, New York, NY 10010, USA.
Brain. 2025 May 13;148(5):1639-1651. doi: 10.1093/brain/awae341.
Parkinson's disease is a progressive neurodegenerative disease with well-documented motor symptoms and less recognized, but significant, non-motor symptoms. These non-motor symptoms include prodromal pain and peripheral neuropathy, the causes of which are unknown. We investigated the role of DJ-1/PARK7, a Parkinson's disease-associated gene, in prodromal pain and peripheral neuropathy. Using Dj-1-deficient mice, we conducted comprehensive sensory tests, cutaneous staining, molecular analyses and electrophysiological studies on mouse and human primary sensory neurons from dorsal root ganglia. We found that these mice exhibited cold hypersensitivity, oxidative stress, and neuropathy of the cutaneous fibres of primary sensory neurons before any motor impairments were observed. Mechanistically, DJ-1 in primary sensory neurons regulated this hypersensitivity and neuropathy via TRPA1 signalling. Interestingly, we discovered that DJ-1 also plays a role in the progression of chemotherapy-induced peripheral neuropathies. Pain and mechanisms associated with these neuropathies were exacerbated in Dj-1-deficient mice but were significantly reduced by the pharmacological activation of Dj-1. Importantly, we also confirmed the expression of DJ-1 and its therapeutic potential in human primary sensory neurons. Thus, we uncover a peripheral mechanism of DJ-1 and propose that it might serve as a new target for developing therapeutic approaches for Parkinson's disease-linked and other painful neuropathies.
帕金森病是一种进行性神经退行性疾病,其运动症状有充分记录,而非运动症状虽鲜为人知但却很显著。这些非运动症状包括前驱性疼痛和周围神经病变,其病因尚不清楚。我们研究了帕金森病相关基因DJ-1/PARK7在前驱性疼痛和周围神经病变中的作用。我们使用DJ-1基因缺陷小鼠,对来自背根神经节的小鼠和人类初级感觉神经元进行了全面的感觉测试、皮肤染色、分子分析和电生理研究。我们发现,在观察到任何运动障碍之前,这些小鼠就表现出冷超敏反应、氧化应激以及初级感觉神经元皮肤纤维的神经病变。从机制上讲,初级感觉神经元中的DJ-1通过TRPA1信号通路调节这种超敏反应和神经病变。有趣的是,我们发现DJ-1在化疗诱导的周围神经病变的进展中也起作用。DJ-1基因缺陷小鼠中与这些神经病变相关的疼痛和机制加剧,但通过DJ-1的药理学激活可显著减轻。重要的是,我们还证实了DJ-1在人类初级感觉神经元中的表达及其治疗潜力。因此,我们揭示了DJ-1的一种外周机制,并提出它可能作为开发针对帕金森病相关及其他疼痛性神经病变治疗方法的新靶点。