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Since the antioxidant response lack could depend on the hypoacetylation of genes coding for detoxifying enzymes, we treated lymphoblasts and fibroblasts mutated for the FANC-A gene with some histone deacetylase inhibitors (HDACi), namely, valproic acid (VPA), beta-hydroxybutyrate (OHB), and EX527 (a Sirt1 inhibitor), under basal conditions and after hydrogen peroxide addition. The results show that VPA increased catalase and glutathione reductase expression and activity, corrected the metabolic defect, lowered lipid peroxidation, restored the mitochondrial fusion and fission balance, and improved mitomycin survival. In contrast, OHB, despite a slight increase in antioxidant enzyme expressions, exacerbated the metabolic defect, increasing oxidative stress production, probably because it also acts as an oxidative phosphorylation metabolite, while EX527 showed no effect. In conclusion, the data suggest that VPA could be a promising drug to modulate the gene expression in FA cells, confirming that the antioxidant response modulation plays a pivotal in FA pathogenesis as it acts on both oxidative stress levels and the mitochondrial metabolism and dynamics quality.<\/jats:p>","DOI":"10.3390\/antiox12051100","type":"journal-article","created":{"date-parts":[[2023,5,16]],"date-time":"2023-05-16T02:20:13Z","timestamp":1684203613000},"page":"1100","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Effects of Deacetylase Inhibition on the Activation of the Antioxidant Response and Aerobic Metabolism in Cellular Models of Fanconi Anemia"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4591-3260","authenticated-orcid":false,"given":"Nadia","family":"Bertola","sequence":"first","affiliation":[{"name":"Department of Experimental Medicine, University of Genoa, Via De Toni 14, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7723-2294","authenticated-orcid":false,"given":"Stefano","family":"Regis","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical and Experimental Immunology, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Bruno","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine, University of Genoa, Via De Toni 14, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2167-8817","authenticated-orcid":false,"given":"Andrea Nicola","family":"Mazzarello","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine, University of Genoa, Via De Toni 14, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Serra","sequence":"additional","affiliation":[{"name":"Laboratory of Clinical and Experimental Immunology, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4712-3708","authenticated-orcid":false,"given":"Michela","family":"Lupia","sequence":"additional","affiliation":[{"name":"Haematology Unit, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Sabatini","sequence":"additional","affiliation":[{"name":"Stem Cell Laboratory and Cell Therapy Center, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Corsolini","sequence":"additional","affiliation":[{"name":"Centro di Diagnostica Genetica e Biochimica delle Malattie Metaboliche, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0803-1042","authenticated-orcid":false,"given":"Silvia","family":"Ravera","sequence":"additional","affiliation":[{"name":"Department of Experimental Medicine, University of Genoa, Via De Toni 14, 16132 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrico","family":"Cappelli","sequence":"additional","affiliation":[{"name":"Haematology Unit, IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16148 Genova, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.mrfmmm.2008.11.004","article-title":"The Genetic and Molecular Basis of Fanconi Anemia","volume":"668","author":"Joenje","year":"2009","journal-title":"Mutat. Res."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3759","DOI":"10.1182\/blood-2010-08-299917","article-title":"Origin, Functional Role, and Clinical Impact of Fanconi Anemia FANCA Mutations","volume":"117","author":"Castella","year":"2011","journal-title":"Blood"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1038\/sj.onc.1207256","article-title":"Inactivation of the Fanconi Anemia\/BRCA Pathway in Lung and Oral Cancers: Implications for Treatment and Survival","volume":"23","author":"Marsit","year":"2004","journal-title":"Oncogene"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/j.oraloncology.2018.10.016","article-title":"Aerobic Metabolism Dysfunction as One of the Links between Fanconi Anemia-Deficient Pathway and the Aggressive Cell Invasion in Head and Neck Cancer Cells","volume":"87","author":"Cappelli","year":"2018","journal-title":"Oral Oncol."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Del Valle, J., Rofes, P., Moreno-Cabrera, J.M., L\u00f3pez-D\u00f3riga, A., Belhadj, S., Vargas-Parra, G., Teul\u00e9, \u00c0., Cuesta, R., Mu\u00f1oz, X., and Campos, O. (2020). Exploring the Role of Mutations in Fanconi Anemia Genes in Hereditary Cancer Patients. Cancers, 12.","DOI":"10.3390\/cancers12040829"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Garc\u00eda-De-teresa, B., Rodr\u00edguez, A., and Frias, S. (2020). Chromosome Instability in Fanconi Anemia: From Breaks to Phenotypic Consequences. Genes, 11.","DOI":"10.20944\/preprints202011.0563.v2"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"2253","DOI":"10.1093\/hmg\/10.20.2253","article-title":"Fanconi Anemia and DNA Repair","volume":"10","author":"Grompe","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e24048","DOI":"10.4161\/rdis.24048","article-title":"Damaged Mitochondria and Overproduction of ROS in Fanconi Anemia Cells","volume":"1","author":"Lyakhovich","year":"2013","journal-title":"Rare Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.freeradbiomed.2013.01.015","article-title":"From Clinical Description, to in Vitro and Animal Studies, and Backward to Patients: Oxidative Stress and Mitochondrial Dysfunction in Fanconi Anemia","volume":"58","author":"Pagano","year":"2013","journal-title":"Free Radic. Biol. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1038\/onc.2012.583","article-title":"Evidence of Mitochondrial Dysfunction and Impaired ROS Detoxifying Machinery in Fanconi Anemia Cells","volume":"33","author":"Kumari","year":"2014","journal-title":"Oncogene"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.molmed.2013.07.008","article-title":"Mitochondrial Respiratory Complex I Defects in Fanconi Anemia","volume":"19","author":"Cappelli","year":"2013","journal-title":"Trends Mol. Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1016\/j.biochi.2013.06.006","article-title":"Mitochondrial Respiratory Chain Complex i Defects in Fanconi Anemia Complementation Group A","volume":"95","author":"Ravera","year":"2013","journal-title":"Biochimie"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"101618","DOI":"10.1016\/j.redox.2020.101618","article-title":"The Passage from Bone Marrow Niche to Bloodstream Triggers the Metabolic Impairment in Fanconi Anemia Mononuclear Cells","volume":"36","author":"Cappelli","year":"2020","journal-title":"Redox Biol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1016\/j.bbadis.2017.03.008","article-title":"Defects in Mitochondrial Energetic Function Compels Fanconi Anaemia Cells to Glycolytic Metabolism","volume":"1863","author":"Cappelli","year":"2017","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1111\/bjh.15171","article-title":"Altered Lipid Metabolism Could Drive the Bone Marrow Failure in Fanconi Anaemia","volume":"184","author":"Ravera","year":"2019","journal-title":"Br. J. Haematol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1909","DOI":"10.1089\/ars.2008.2129","article-title":"Oxidative Stress in Fanconi Anemia Hematopoiesis and Disease Progression","volume":"10","author":"Du","year":"2008","journal-title":"Antioxid. Redox Signal."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Cappelli, E., Bertola, N., Bruno, S., Degan, P., Regis, S., Corsolini, F., Banelli, B., Dufour, C., and Ravera, S. (2021). A Multidrug Approach to Modulate the Mitochondrial Metabolism Impairment and Relative Oxidative Stress in Fanconi Anemia Complementation Group A. Metabolites, 12.","DOI":"10.3390\/metabo12010006"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.neulet.2016.10.032","article-title":"Homocysteine Inhibits Neural Stem Cells Survival by Inducing DNA Interstrand Cross-Links via Oxidative Stress","volume":"635","author":"Wang","year":"2016","journal-title":"Neurosci. Lett."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1","DOI":"10.18388\/abp.2006_3359","article-title":"Regulatory Mechanisms of Gene Expression: Complexity with Elements of Deterministic Chaos","volume":"53","author":"Jura","year":"2006","journal-title":"Acta Biochim. Polonica"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1139\/o05-041","article-title":"Role of Histone Acetylation in the Control of Gene Expression","volume":"83","author":"Verdone","year":"2005","journal-title":"Biochem. Cell Biol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"371832","DOI":"10.1155\/2011\/371832","article-title":"Physical and Functional HAT\/HDAC Interplay Regulates Protein Acetylation Balance","volume":"2011","author":"Peserico","year":"2011","journal-title":"J. Biomed. Biotechnol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1372","DOI":"10.1016\/j.bbapap.2016.06.007","article-title":"The World of Protein Acetylation","volume":"1864","author":"Drazic","year":"2016","journal-title":"Biochim. Biophys. Acta BBA Proteins Proteomics"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/s12276-020-0382-4","article-title":"A Short Guide to Histone Deacetylases Including Recent Progress on Class II Enzymes","volume":"52","author":"Park","year":"2020","journal-title":"Exp. Mol. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1615\/CritRevOncog.2015012997","article-title":"Histone Deacetylases and Mechanisms of Regulation of Gene Expression (Histone Deacetylases in Cancer)","volume":"20","author":"Chen","year":"2015","journal-title":"Crit. Rev. Oncog."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1158\/1541-7786.MCR-07-0324","article-title":"Histone Deacetylase Inhibitors: Overview and Perspectives","volume":"5","author":"Dokmanovic","year":"2007","journal-title":"Mol. Cancer Res."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1038\/sj.bjp.0707166","article-title":"HDAC Inhibitors as Anti-Inflammatory Agents","volume":"150","author":"Adcock","year":"2007","journal-title":"Br. J. Pharmacol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1111\/1759-7714.12974","article-title":"Targeted Inhibition of Histone Deacetylase 6 in Inflammatory Diseases","volume":"10","author":"Ran","year":"2019","journal-title":"Thorac. Cancer"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"151","DOI":"10.3389\/fimmu.2019.00151","article-title":"Restoring T Cell Tolerance, Exploring the Potential of Histone Deacetylase Inhibitors for the Treatment of Juvenile Idiopathic Arthritis","volume":"10","author":"Nijhuis","year":"2019","journal-title":"Front. Immunol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"2718","DOI":"10.4049\/jimmunol.0901467","article-title":"Histone Deacetylase Inhibitors Suppress Inflammatory Activation of Rheumatoid Arthritis Patient Synovial Macrophages and Tissue","volume":"184","author":"Grabiec","year":"2010","journal-title":"J. Immunol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"679655","DOI":"10.3389\/fendo.2021.679655","article-title":"Histone Deacetylases in the Pathogenesis of Diabetic Cardiomyopathy","volume":"12","author":"Ke","year":"2021","journal-title":"Front. Endocrinol."},{"key":"ref_31","first-page":"1004","article-title":"The Roles of Histone Deacetylases and Their Inhibitors in Cancer Therapy","volume":"8","author":"Li","year":"2020","journal-title":"Front. Cell Dev. Biol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1312","DOI":"10.1038\/cdd.2016.9","article-title":"Histone Deacetylase Inhibition Reveals a Tumor-Suppressive Function of MYC-Regulated MiRNA in Breast and Lung Carcinoma","volume":"23","author":"Adams","year":"2016","journal-title":"Cell Death Differ."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"108","DOI":"10.3389\/fonc.2015.00108","article-title":"The Role of Histone Acetyltransferases in Normal and Malignant Hematopoiesis","volume":"5","author":"Sun","year":"2015","journal-title":"Front. Oncol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1186\/s40170-021-00240-1","article-title":"SIRT6 Enhances Oxidative Phosphorylation in Breast Cancer and Promotes Mammary Tumorigenesis in Mice","volume":"9","author":"Becherini","year":"2021","journal-title":"Cancer Metab."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1038\/nrd4360","article-title":"Histone Deacetylases and Their Inhibitors in Cancer, Neurological Diseases and Immune Disorders","volume":"13","author":"Falkenberg","year":"2014","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"537","DOI":"10.3389\/fphar.2020.00537","article-title":"Histone Deacetylases Inhibitors in Neurodegenerative Diseases, Neuroprotection and Neuronal Differentiation","volume":"11","author":"Shukla","year":"2020","journal-title":"Front. Pharmacol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"648","DOI":"10.1093\/nar\/gkv1019","article-title":"Impaired TIP60-Mediated H4K16 Acetylation Accounts for the Aberrant Chromatin Accumulation of 53BP1 and RAP80 in Fanconi Anemia Pathway-Deficient Cells","volume":"44","author":"Renaud","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"ref_38","first-page":"10017","article-title":"The Multifaceted Influence of Histone Deacetylases on DNA Damage Signalling and DNA Repair","volume":"44","author":"Roos","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.leukres.2016.03.007","article-title":"Histone Deacetylase Inhibitors Decrease NHEJ Both by Acetylation of Repair Factors and Trapping of PARP1 at DNA Double-Strand Breaks in Chromatin","volume":"45","author":"Robert","year":"2016","journal-title":"Leuk. Res."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"669","DOI":"10.2165\/00023210-200216100-00003","article-title":"Basic Pharmacology of Valproate: A Review after 35 Years of Clinical Use for the Treatment of Epilepsy","volume":"16","year":"2002","journal-title":"CNS Drugs"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1158\/0008-5472.CAN-03-0799","article-title":"Histone Deacetylase Is a Target of Valproic Acid-Mediated Cellular Differentiation","volume":"64","author":"Gurvich","year":"2004","journal-title":"Cancer Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1038\/nrd2133","article-title":"Anticancer Activities of Histone Deacetylase Inhibitors","volume":"5","author":"Bolden","year":"2006","journal-title":"Nat. Rev. Drug Disc."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1126\/science.1227166","article-title":"Suppression of Oxidative Stress by \u03b2-Hydroxybutyrate, an Endogenous Histone Deacetylase Inhibitor","volume":"339","author":"Shimazu","year":"2013","journal-title":"Science"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1080\/14756366.2020.1758691","article-title":"Biochemical Mechanism and Biological Effects of the Inhibition of Silent Information Regulator 1 (SIRT1) by EX527 (SEN0014196 or Selisistat)","volume":"35","author":"Broussy","year":"2020","journal-title":"J. Enzym. Inhib. Med. Chem."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"30661","DOI":"10.18632\/oncotarget.25771","article-title":"SIRT1-Dependent Epigenetic Regulation of H3 and H4 Histone Acetylation in Human Breast Cancer","volume":"9","author":"Judes","year":"2018","journal-title":"Oncotarget"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1016\/j.tibs.2010.07.003","article-title":"Sirtuin Regulation of Mitochondria: Energy Production, Apoptosis, and Signaling","volume":"35","author":"Verdin","year":"2010","journal-title":"Trends Biochem. Sci."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1158\/0008-5472.CAN-04-3063","article-title":"Histone Deacetylase Inhibitor Valproic Acid Enhances the Cytokine-Induced Expansion of Human Hematopoietic Stem Cells","volume":"65","author":"Tatarelli","year":"2005","journal-title":"Cancer Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jphs.2019.10.007","article-title":"\u03b2-Hydroxybutyrate Enhances the Cytotoxic Effect of Cisplatin via the Inhibition of HDAC\/Survivin Axis in Human Hepatocellular Carcinoma Cells","volume":"142","author":"Mikami","year":"2020","journal-title":"J. Pharmacol. Sci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/S0301-472X(02)00782-8","article-title":"Phenotypic Correction of Primary Fanconi Anemia T Cells with Retroviral Vectors as a Diagnostic Tool","volume":"30","author":"Hanenberg","year":"2002","journal-title":"Exp. Hematol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1016\/0003-2697(76)90527-3","article-title":"A Rapid and Sensitive Method for the Quantitation of Microgram Quantities of Protein Utilizing the Principle of Protein-Dye Binding","volume":"72","author":"Bradford","year":"1976","journal-title":"Anal. Biochem."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1093\/carcin\/bgy076","article-title":"Curcumin Induces a Fatal Energetic Impairment in Tumor Cells in Vitro and in Vivo by Inhibiting ATP-Synthase Activity","volume":"39","author":"Bianchi","year":"2018","journal-title":"Carcinogenesis"},{"key":"ref_52","doi-asserted-by":"crossref","unstructured":"Bertola, N., Degan, P., Cappelli, E., and Ravera, S. (2022). Mutated FANCA Gene Role in the Modulation of Energy Metabolism and Mitochondrial Dynamics in Head and Neck Squamous Cell Carcinoma. Cells, 11.","DOI":"10.3390\/cells11152353"},{"key":"ref_53","first-page":"1952","article-title":"Mitochondrial Aldehyde Dehydrogenase-2 Activation Prevents \u03b2-Amyloid-Induced Endothelial Cell Dysfunction and Restores Angiogenesis","volume":"126","author":"Solito","year":"2013","journal-title":"J. Cell Sci."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"70715","DOI":"10.18632\/oncotarget.12209","article-title":"Glutathione-Mediated Antioxidant Response and Aerobic Metabolism: Two Crucial Factors Involved in Determining the Multi-Drug Resistance of High-Risk Neuroblastoma","volume":"7","author":"Colla","year":"2016","journal-title":"Oncotarget"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2021\/6626286","article-title":"Photobiomodulation and Oxidative Stress: 980 Nm Diode Laser Light Regulates Mitochondrial Activity and Reactive Oxygen Species Production","volume":"2021","author":"Amaroli","year":"2021","journal-title":"Oxid. Med. Cell Longev."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"25441","DOI":"10.1038\/srep25441","article-title":"Evaluation of Energy Metabolism and Calcium Homeostasis in Cells Affected by Shwachman-Diamond Syndrome","volume":"6","author":"Ravera","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"16519","DOI":"10.1038\/s41598-020-73594-z","article-title":"Berberine Affects Mitochondrial Activity and Cell Growth of Leukemic Cells from Chronic Lymphocytic Leukemia Patients","volume":"10","author":"Ravera","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.bbabio.2004.09.004","article-title":"P\/O Ratios of Mitochondrial Oxidative Phosphorylation","volume":"1706","author":"Hinkle","year":"2005","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"321","DOI":"10.15252\/embj.201696380","article-title":"Miro Proteins Coordinate Microtubule- and Actin-Dependent Mitochondrial Transport and Distribution","volume":"37","author":"Ivankovic","year":"2018","journal-title":"EMBO J."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"e639","DOI":"10.1002\/mgg3.639","article-title":"Two Further Patients with Warsaw Breakage Syndrome. Is a Mild Phenotype Possible?","volume":"7","author":"Bottega","year":"2019","journal-title":"Mol. Genet. Genomic Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1038\/s41431-017-0046-6","article-title":"Fanconi Anemia: From DNA Repair to Metabolism","volume":"26","author":"Ravera","year":"2018","journal-title":"Eur. J. Hum. Genet."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/j.molmed.2019.01.003","article-title":"New Insights and Perspectives in Fanconi Anemia Research","volume":"25","author":"Degan","year":"2019","journal-title":"Trends Mol. Med."},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Bertola, N., Bruno, S., Capanni, C., Columbaro, M., Mazzarello, A.N., Corsolini, F., Regis, S., Degan, P., Cappelli, E., and Ravera, S. (2023). Altered Mitochondrial Dynamic in Lymphoblasts and Fibroblasts Mutated for FANCA-A Gene: The Central Role of DRP1. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms24076557"},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Columbaro, M., Ravera, S., Capanni, C., Panfoli, I., Cuccarolo, P., Stroppiana, G., Degan, P., and Cappell, E. (2014). Treatment of FANCA Cells with Resveratrol and N-Acetylcysteine: A Comparative Study. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0104857"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1111\/j.1600-0609.2005.00507.x","article-title":"Oxidative Stress as a Multiple Effector in Fanconi Anaemia Clinical Phenotype","volume":"75","author":"Pagano","year":"2005","journal-title":"Eur. J. Haematol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"205","DOI":"10.4103\/2141-9248.157511","article-title":"Oxidative Stress-a Phenotypic Hallmark of Fanconi Anemia and Down Syndrome: The Effect of Antioxidants","volume":"5","author":"Afifi","year":"2015","journal-title":"Ann. Med. Health Sci. Res."},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Juan, C.A., de la Lastra, J.M.P., Plou, F.J., and P\u00e9rez-Lebe\u00f1a, E. (2021). The Chemistry of Reactive Oxygen Species (ROS) Revisited: Outlining Their Role in Biological Macromolecules (DNA, Lipids and Proteins) and Induced Pathologies. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22094642"},{"key":"ref_68","first-page":"363","article-title":"Control Mechanisms in Mitochondrial Oxidative Phosphorylation","volume":"8","year":"2013","journal-title":"Neural. Regen. Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.tem.2015.12.001","article-title":"Mitochondrial Dynamics and Metabolic Regulation","volume":"27","author":"Wai","year":"2016","journal-title":"Trends Endocrinol. Metab."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1007\/s00018-017-2665-z","article-title":"Mesenchymal Stem Cells from Preterm to Term Newborns Undergo a Significant Switch from Anaerobic Glycolysis to the Oxidative Phosphorylation","volume":"75","author":"Ravera","year":"2018","journal-title":"Cell Mol. Life Sci."},{"key":"ref_71","doi-asserted-by":"crossref","unstructured":"Yang, D., and Kim, J. (2019). Mitochondrial Retrograde Signalling and Metabolic Alterations in the Tumour Microenvironment. Cells, 8.","DOI":"10.3390\/cells8030275"},{"key":"ref_72","first-page":"1","article-title":"Nuclear Localization of Mitochondrial TCA Cycle Enzymes Modulates Pluripotency via Histone Acetylation","volume":"13","author":"Li","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1038\/ncb3529","article-title":"The Mitochondrial Respiratory Chain Is Essential for Haematopoietic Stem Cell Function","volume":"19","author":"Weinberg","year":"2017","journal-title":"Nat. Cell Biol."}],"container-title":["Antioxidants"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3921\/12\/5\/1100\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:35:35Z","timestamp":1760124935000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3921\/12\/5\/1100"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,15]]},"references-count":73,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2023,5]]}},"alternative-id":["antiox12051100"],"URL":"https:\/\/doi.org\/10.3390\/antiox12051100","relation":{},"ISSN":["2076-3921"],"issn-type":[{"value":"2076-3921","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,15]]}}}