Rey Federica, Berardo Clarissa, Maghraby Erika, Mauri Alessia, Messa Letizia, Esposito Letizia, Casili Giovanna, Ottolenghi Sara, Bonaventura Eleonora, Cuzzocrea Salvatore, Zuccotti Gianvincenzo, Tonduti Davide, Esposito Emanuela, Paterniti Irene, Cereda Cristina, Carelli Stephana
Pediatric Clinical Research Center "Romeo ed Enrica Invernizzi", Department of Biomedical and Clinical Sciences, University of Milano, 20157 Milano, Italy.
Center of Functional Genomics and Rare diseases, Department of Pediatrics, Buzzi Children's Hospital, 20154 Milano, Italy.
Antioxidants (Basel). 2023 Apr 20;12(4):965. doi: 10.3390/antiox12040965.
Oxygen is a central molecule for numerous metabolic and cytophysiological processes, and, indeed, its imbalance can lead to numerous pathological consequences. In the human body, the brain is an aerobic organ and for this reason, it is very sensitive to oxygen equilibrium. The consequences of oxygen imbalance are especially devastating when occurring in this organ. Indeed, oxygen imbalance can lead to hypoxia, hyperoxia, protein misfolding, mitochondria dysfunction, alterations in heme metabolism and neuroinflammation. Consequently, these dysfunctions can cause numerous neurological alterations, both in the pediatric life and in the adult ages. These disorders share numerous common pathways, most of which are consequent to redox imbalance. In this review, we will focus on the dysfunctions present in neurodegenerative disorders (specifically Alzheimer's disease, Parkinson's disease and amyotrophic lateral sclerosis) and pediatric neurological disorders (X-adrenoleukodystrophies, spinal muscular atrophy, mucopolysaccharidoses and Pelizaeus-Merzbacher Disease), highlighting their underlining dysfunction in redox and identifying potential therapeutic strategies.
氧气是众多代谢和细胞生理过程的核心分子,事实上,其失衡会导致众多病理后果。在人体中,大脑是一个需氧器官,因此,它对氧平衡非常敏感。当氧失衡发生在这个器官时,其后果尤其具有破坏性。确实,氧失衡会导致缺氧、高氧、蛋白质错误折叠、线粒体功能障碍、血红素代谢改变和神经炎症。因此,这些功能障碍会在儿童期和成年期引发众多神经改变。这些疾病有许多共同的途径,其中大多数是氧化还原失衡的结果。在这篇综述中,我们将聚焦于神经退行性疾病(特别是阿尔茨海默病、帕金森病和肌萎缩侧索硬化症)和儿童神经系统疾病(X-肾上腺脑白质营养不良、脊髓性肌萎缩症、黏多糖贮积症和佩利措伊斯-梅茨巴赫病)中存在的功能障碍,突出它们在氧化还原方面的潜在功能障碍,并确定潜在的治疗策略。