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Mitophagy in neurodegenerative diseases
Neurochemistry international ( IF 4.4 ) Pub Date : 2017-08-08 , DOI: 10.1016/j.neuint.2017.08.004
Carlo Rodolfo , Silvia Campello , Francesco Cecconi

Neurodegenerative diseases, such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD), and Amyotrophic Lateral Sclerosis (ALS), are a complex “family” of pathologies, characterised by the progressive loss of neurons and/or neuronal functions, leading to severe physical and cognitive inabilities in affected patients. These syndromes, despite differences in the causative events, the onset, and the progression of the disease, share as common features the presence of aggregate-prone neuro-toxic proteins, in the form of aggresomes and/or inclusion bodies, perturbing cellular homeostasis and neuronal function (Popovic et al., 2014), and the presence of dysfunctional mitochondria. The removal of protein aggregates and of damaged organelles, through the ubiquitin-proteasome system (UPS) and/or the autophagy/lysosome machinery, is a crucial step for the maintenance of neuronal homeostasis. Indeed, their impairment has been reported as associated with the development of these diseases. In this review, we focus on the role played by mitophagy, a specialised form of autophagy, in the onset and progression of major neurodegenerative diseases, as well as on possible therapeutic approaches involving mitophagy modulation.



中文翻译:

神经退行性疾病中的线粒体吞噬

神经退行性疾病,例如帕金森氏病(PD),阿尔茨海默氏病(AD),亨廷顿氏病(HD)和肌萎缩性侧索硬化症(ALS),是一个复杂的病理“家族”,其特征是神经元和/或神经元的逐渐丧失神经功能,导致受影响的患者严重的身体和认知能力下降。这些综合征尽管在病因,发病和进展方面存在差异,但它们的共同特点是存在聚集体倾向的神经毒性蛋白,以聚集体和/或包涵体的形式存在,扰动细胞的稳态和代谢。神经元功能(Popovic等,2014),以及线粒体功能异常。去除蛋白质聚集体和受损细胞器,通过泛素-蛋白酶体系统(UPS)和/或自噬/溶酶体机制,是维持神经元稳态的关键步骤。实际上,已经报道了它们的损害与这些疾病的发展有关。在这篇综述中,我们集中于自噬的一种特殊形式-自噬在主要神经退行性疾病的发作和发展中所扮演的角色,以及涉及自噬调节的可能治疗方法。

更新日期:2017-08-08
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