Reducing Mitochondrial ROS Improves Disease-related Pathology in a Mouse Model of Ataxia-telangiectasia
D'Souza AD, Parish IA, Krause DS, Kaech SM, Shadel GS. Reducing Mitochondrial ROS Improves Disease-related Pathology in a Mouse Model of Ataxia-telangiectasia. Molecular Therapy 2012, 21: 42-48. PMID: 23011031, PMCID: PMC3538311, DOI: 10.1038/mt.2012.203.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAtaxia TelangiectasiaAtaxia Telangiectasia Mutated ProteinsCatalaseCD8-Positive T-LymphocytesCell Cycle ProteinsDisease Models, AnimalDNA-Binding ProteinsHematopoiesisImmunologic MemoryLymphomaMiceMice, KnockoutMitochondriaProtein Serine-Threonine KinasesReactive Oxygen SpeciesThymus NeoplasmsTumor Suppressor ProteinsConceptsMitochondrial reactive oxygen speciesReactive oxygen speciesAtaxia telangiectasiaT cell developmental defectsDNA damage responseDisease ataxia telangiectasiaMitochondrial ROS productionOverexpression of catalaseATM kinaseRedox sensingDevelopmental defectsLatter phenotypePartial rescueBone marrow hematopoiesisCancer predispositionNull mouse modelMitochondrial dysfunctionMacrophage differentiationTORC1ROS productionCancer developmentOxygen speciesMouse modelTS pathologyMarrow hematopoiesis