ArticleNeurochemical research2026
Ethylmalonic and Methylsuccinic Acids Disrupt Bioenergetics and Induce Mitochondrial Permeability Transition Through Thiol Redox Modulation in Rat Striatum: Potential Mechanisms Involved in Ethylmalonic Encephalopathy.
Article in Neurochemical research, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Ethylmalonic (EMA) and methylsuccinic (MSA) acids concentrations are elevated in tissues and body fluids of patients with ethylmalonic encephalopathy (EE), a mitochondrial disorder associated with basal ganglia abnormalities. To clarify the pathophysiology of this disorder, we evaluated the effects of EMA and MSA on bioenergetics, redox homeostasis and mitochondrial permeability transition (MPT) in rat striatum. We verified that EMA and MSA reduced state 3 and uncoupled respiration and inhibited the activities of α-ketoglutarate dehydrogenase and complex IV of the electron transport chain. Both organic acids also decreased mitochondrial membrane potential and Ca
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