The article discusses the effects of manganese superoxide dismutase (SOD2) deficiency in mice, highlighting the resulting neurodegeneration, myocardial injury, and perinatal death. SOD2 is crucial for converting superoxide radicals into oxygen and hydrogen peroxide, serving as the primary defense against mitochondrial superoxide. The study presents a line of SOD2 knockout mice (SOD2m1BCM/SOD2m1BCM) that survive up to 3 weeks, exhibiting severe anemia, neuronal degeneration in the basal ganglia and brainstem, and progressive motor disturbances. These mice also show mitochondrial injury in neurons and cardiac myocytes, with approximately 10% displaying enlarged and dilated hearts. Unlike a previously described model where homozygotes die within 5 days, these mice show distinct pathologies, including central nervous system injury and motor disturbances. The research indicates that SOD2 deficiency increases susceptibility to oxidative mitochondrial injury in metabolically active tissues. The study also notes that SOD2-deficient mice exhibit reduced growth rates, histological abnormalities, and motor deficits. The findings suggest that SOD2 plays a critical role in protecting against oxidative damage, particularly in the central nervous system and heart. The research provides insights into the role of SOD2 in various diseases and highlights the importance of understanding its function in mitochondrial health.The article discusses the effects of manganese superoxide dismutase (SOD2) deficiency in mice, highlighting the resulting neurodegeneration, myocardial injury, and perinatal death. SOD2 is crucial for converting superoxide radicals into oxygen and hydrogen peroxide, serving as the primary defense against mitochondrial superoxide. The study presents a line of SOD2 knockout mice (SOD2m1BCM/SOD2m1BCM) that survive up to 3 weeks, exhibiting severe anemia, neuronal degeneration in the basal ganglia and brainstem, and progressive motor disturbances. These mice also show mitochondrial injury in neurons and cardiac myocytes, with approximately 10% displaying enlarged and dilated hearts. Unlike a previously described model where homozygotes die within 5 days, these mice show distinct pathologies, including central nervous system injury and motor disturbances. The research indicates that SOD2 deficiency increases susceptibility to oxidative mitochondrial injury in metabolically active tissues. The study also notes that SOD2-deficient mice exhibit reduced growth rates, histological abnormalities, and motor deficits. The findings suggest that SOD2 plays a critical role in protecting against oxidative damage, particularly in the central nervous system and heart. The research provides insights into the role of SOD2 in various diseases and highlights the importance of understanding its function in mitochondrial health.