title: Oxidative Stress in Cancer-Prone Genetic Diseases in Pediatric Age: The Role of Mitochondrial Dysfunction.
abstract: Oxidative stress is a distinctive sign in several genetic disorders characterized by cancer predisposition, such as Ataxia-Telangiectasia, Fanconi Anemia, Down syndrome, progeroid syndromes, Beckwith-Wiedemann syndrome, and Costello syndrome. Recent literature unveiled new molecular mechanisms linking oxidative stress to the pathogenesis of these conditions, with particular regard to mitochondrial dysfunction. Since mitochondria are one of the major sites of ROS production as well as one of the major targets of their action, this dysfunction is thought to be the cause of the prooxidant status. Deeper insight of the pathogenesis of the syndromes raises the possibility to identify new possible therapeutic targets. In particular, the use of mitochondrial-targeted agents seems to be an appropriate clinical strategy in order to improve the quality of life and the life span of the patients.
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy.
authors.forename: Serafina
authors.identifier: 0000-0001-5478-5657
authors.initials: S
authors.lastname: Perrone
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy.
authors.forename: Federica
authors.identifier: 0000-0002-0395-7524
authors.initials: F
authors.lastname: Lotti
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy.
authors.forename: Ursula
authors.identifier: 0000-0002-7500-8686
authors.initials: U
authors.lastname: Geronzi
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy.
authors.forename: Elisa
authors.identifier: 0000-0002-0878-5346
authors.initials: E
authors.lastname: Guidoni
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy; UOC Patologia Clinica, AOUS Siena, 53100 Siena, Italy.
authors.forename: Mariangela
authors.initials: M
authors.lastname: Longini
authors.affiliation: Department of Molecular and Developmental Medicine, University of Siena, 53100 Siena, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Buonocore
mesh_terms: D000367:Age Factors; D000818:Animals; D016588:Anticarcinogenic Agents / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism*; D002471:Cell Transformation, Neoplastic / Q000235:genetics / Q000378:metabolism; D020022:Genetic Predisposition to Disease; D006801:Humans; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism*; D028361:Mitochondrial Diseases / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism*; D009369:Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000517:prevention & control; D018384:Oxidative Stress / Q000187:drug effects; D011218:Prader-Willi Syndrome / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism*; D012307:Risk Factors
chemical_list: D016588:Anticarcinogenic Agents; D000975:Antioxidants