Redox Imbalance in Neurological Disorders in Adults and Children

Author:

Rey Federica12ORCID,Berardo Clarissa12ORCID,Maghraby Erika13,Mauri Alessia12ORCID,Messa Letizia24,Esposito Letizia12ORCID,Casili Giovanna5,Ottolenghi Sara6ORCID,Bonaventura Eleonora78,Cuzzocrea Salvatore5ORCID,Zuccotti Gianvincenzo19ORCID,Tonduti Davide178,Esposito Emanuela5ORCID,Paterniti Irene5,Cereda Cristina2ORCID,Carelli Stephana12ORCID

Affiliation:

1. Pediatric Clinical Research Center “Romeo ed Enrica Invernizzi”, Department of Biomedical and Clinical Sciences, University of Milano, 20157 Milano, Italy

2. Center of Functional Genomics and Rare diseases, Department of Pediatrics, Buzzi Children’s Hospital, 20154 Milano, Italy

3. Department of Biology and Biotechnology “L. Spallanzani”, University of Pavia, 27100 Pavia, Italy

4. Department of Electronics, Information and Bioengineering (DEIB), Politecnico di Milano, 20133 Milano, Italy

5. Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, 98166 Messina, Italy

6. Department of Medicine and Surgery, University of Milano Bicocca, 20126 Milano, Italy

7. Child Neurology Unit, Buzzi Children’s Hospital, 20154 Milano, Italy

8. Center for Diagnosis and Treatment of Leukodystrophies and Genetic Leukoencephalopathies (COALA), Buzzi Children’s Hospital, 20154 Milano, Italy

9. Department of Pediatrics, Buzzi Children’s Hospital, 20154 Milano, Italy

Abstract

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.

Funder

Italian Ministry of Health

Publisher

MDPI AG

Subject

Cell Biology,Clinical Biochemistry,Molecular Biology,Biochemistry,Physiology

Reference345 articles.

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