Neuroregeneration and Stem Cell Programs, Institute for Cell Engineering, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Department of Neurosurgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Mov Disord. 2024 Apr;39(4):644-650. doi: 10.1002/mds.29748. Epub 2024 Feb 23.
Parkinson's disease (PD) is a progressive neurodegenerative condition that pathognomonically involves the death of dopaminergic neurons in the substantia nigra pars compacta, resulting in a myriad of motor and non-motor symptoms. Given the insurmountable burden of this disease on the population and healthcare system, significant efforts have been put forth toward generating disease modifying therapies. This class of treatments characteristically alters disease course, as opposed to current strategies that focus on managing symptoms. Previous literature has implicated the cell death pathway known as parthanatos in PD progression. Inhibition of this pathway by targeting poly (ADP)-ribose polymerase 1 (PARP1) prevents neurodegeneration in a model of idiopathic PD. However, PARP1 has a vast repertoire of functions within the body, increasing the probability of side effects with the long-term treatment likely necessary for clinically significant neuroprotection. Recent work culminated in the development of a novel agent targeting the macrophage migration inhibitory factor (MIF) nuclease domain, also named parthanatos-associated apoptosis-inducing factor nuclease (PAAN). This nuclease activity specifically executes the terminal step in parthanatos. Parthanatos-associated apoptosis-inducing factor nuclease inhibitor-1 was neuroprotective in multiple preclinical mouse models of PD. This piece will focus on contextualizing this discovery, emphasizing its significance, and discussing its potential implications for parthanatos-directed treatment. © 2024 International Parkinson and Movement Disorder Society.
帕金森病(PD)是一种进行性神经退行性疾病,其特征性地涉及黑质致密部多巴胺能神经元的死亡,导致多种运动和非运动症状。鉴于这种疾病给人群和医疗保健系统带来的巨大负担,人们已经做出了巨大的努力来开发疾病修饰疗法。这类治疗方法通常会改变疾病进程,而不是目前专注于管理症状的策略。先前的文献表明,细胞死亡途径中的 parthanatos 与 PD 进展有关。通过靶向聚(ADP-核糖)聚合酶 1(PARP1)抑制该途径可防止特发性 PD 模型中的神经退行性变。然而,PARP1 在体内具有广泛的功能,长期治疗可能需要长期治疗,这增加了副作用的可能性,而长期治疗对临床显著的神经保护作用很有必要。最近的工作最终开发出了一种针对巨噬细胞迁移抑制因子(MIF)核酸酶结构域的新型药物,也称为 parthanatos 相关凋亡诱导因子核酸酶(PAAN)。这种核酸酶活性专门执行 parthanatos 的最后一步。Parthanatos 相关凋亡诱导因子核酸酶抑制剂-1 在多种 PD 的临床前小鼠模型中具有神经保护作用。这篇文章将重点介绍这一发现的背景、强调其意义,并讨论其对 parthanatos 定向治疗的潜在影响。