da Costa Caiado Maria João, Dolga Amalia M, den Dunnen Wilfred F A
Graduate School of Medical Sciences (GSMS) and Research School of Behavioural and Cognitive Neurosciences (BCN), University of Groningen, 9713 GZ, Groningen, the Netherlands; Department of Pathology and Medical Biology, University Medical Centre Groningen (UMCG), Hanzeplein 1, 9713 GZ, Groningen, the Netherlands; Department of Molecular Pharmacology, Groningen Research Institute of Pharmacy (GRIP), University of Groningen, 9713 AV, Groningen, the Netherlands.
Department of Pathology and Medical Biology, University Medical Centre Groningen (UMCG), Hanzeplein 1, 9713 GZ, Groningen, the Netherlands; Department of Molecular Pharmacology, Groningen Research Institute of Pharmacy (GRIP), University of Groningen, 9713 AV, Groningen, the Netherlands.
Redox Biol. 2025 Feb;79:103478. doi: 10.1016/j.redox.2024.103478. Epub 2024 Dec 19.
Parkinsonian syndromes are characterised by similar motor-related symptomology resulting from dopaminergic neuron damage. While Parkinson's disease (PD) is the most prevalent parkinsonism, we also focus on two other variants, Progressive supranuclear palsy (PSP) and Corticobasal degeneration (CBD). Due to the clinical similarities of these parkinsonisms, and since definite diagnoses are only possible post-mortem, effective therapies and novel biomarkers of disease are scarce. Thus, we explore the current findings relating to the relationship of parkinsonism proteinopathy (α-synuclein in PD, and tau in PSP/CBD) paralleled to a specific form of cell death, ferroptosis. Ferroptosis is characterised by iron-induced lipid peroxidation and several markers of this pathway have been identified to control intracellular iron fluctuations. However, in parkinsonism, these mechanisms are thought to become dysfunctional. Although both proteinopathies have been linked to ferroptosis, much less is known about ferroptotic cell death and tau in the context of PSP/CBD. Interestingly, clinical trials targeting iron have recently shown conflicting results which begs to question the complexity of the ferroptotic pathway and alludes to the need for exploring other ferroptosis-related machinery as possible therapeutic targets. Overall, we address the literature gap in parkinsonism proteinopathy and ferroptosis, and its relevance to understanding disease pathophysiology and aetiology.
帕金森综合征的特征是由多巴胺能神经元损伤导致的类似运动相关症状。虽然帕金森病(PD)是最常见的帕金森症,但我们也关注另外两种变体,进行性核上性麻痹(PSP)和皮质基底节变性(CBD)。由于这些帕金森症在临床上有相似之处,而且只有在死后才能做出明确诊断,因此有效的治疗方法和新的疾病生物标志物很少。因此,我们探讨了与帕金森症蛋白病(PD中的α-突触核蛋白,以及PSP/CBD中的tau蛋白)与一种特定形式的细胞死亡——铁死亡之间关系的当前研究结果。铁死亡的特征是铁诱导的脂质过氧化,并且已经确定了该途径的几个标志物来控制细胞内铁的波动。然而,在帕金森症中,这些机制被认为会出现功能障碍。虽然这两种蛋白病都与铁死亡有关,但在PSP/CBD的背景下,关于铁死亡细胞死亡和tau蛋白的了解要少得多。有趣的是,最近针对铁的临床试验显示出相互矛盾的结果,这就引发了对铁死亡途径复杂性的质疑,并暗示需要探索其他与铁死亡相关的机制作为可能的治疗靶点。总体而言,我们解决了帕金森症蛋白病和铁死亡方面的文献空白,以及其与理解疾病病理生理学和病因学的相关性。