biostudies-literaturePramio JPró-Reitoria de Pesquisa/Universidade Federal do Rio Grande do SulConselho Nacional de Desenvolvimento Científico e TecnológicoFundação de Amparo à Pesquisa do Estado do Rio Grande do SulInstituto Nacional de Ciência e Tecnologia em Excitotoxicidade e NeuroproteçãoInstituto Nacional de Ciência e Tecnologia em Excitotoxicidade e Neuroproteção (INCT-EN)2895-2907https://www.ebi.ac.uk/biostudies/studies/S-EPMC11410132biostudies-literatureUnknown43(6)Isolated sulfite oxidase (ISOD) and molybdenum cofactor (MoCD) deficiencies are genetic diseases biochemically characterized by the toxic accumulation of sulfite in the tissues of patients, including the brain. Neurological dysfunction and brain abnormalities are commonly observed soon after birth, and some patients also have neuropathological alterations in the prenatal period (in utero). Thus, we investigated the effects of sulfite on redox and mitochondrial homeostasis, as well as signaling proteins in the cerebral cortex of rat pups. One-day-old Wistar rats received an intracerebroventricular administration of sulfite (0.5 µmol/g) or vehicle and were euthanized 30 min after injection. Sulfite administration decreased glutathione levels and glutathione S-transferase activity, and increaCellular and molecular neurobiologySulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies.PMC11410132312141/2020-3465671/2014-4402440/2021-816/2551-0000465-0Ribeiro RTBobermin LDWyse ATSPramio Jda Rosa AGSignori MFWajner MMarcuzzo MBGrings MGlanzel NMQuincozes-Santos ALeipnitz GfalseSulfite Impairs Bioenergetics and Redox Status in Neonatal Rat Brain: Insights into the Early Neuropathophysiology of Isolated Sulfite Oxidase and Molybdenum Cofactor Deficiencies.Isolated sulfite oxidase (ISOD) and molybdenum cofactor (MoCD) deficiencies are genetic diseases biochemically characterized by the toxic accumulation of sulfite in the tissues of patients, including the brain. Neurological dysfunction and brain abnormalities are commonly observed soon after birth, and some patients also have neuropathological alterations in the prenatal period (in utero). Thus, we investigated the effects of sulfite on redox and mitochondrial homeostasis, as well as signaling proteins in the cerebral cortex of rat pups. One-day-old Wistar rats received an intracerebroventricular administration of sulfite (0.5 µmol/g) or vehicle and were euthanized 30 min after injection. Sulfite administration decreased glutathione levels and glutathione S-transferase activity, and increa2023-01-01T00:00:00Z2023 Aug2025-04-22T20:10:57.675Z2025-04-06T03:03:24.762ZS-EPMC114101323686224210.1007/s10571-023-01328-6