Ethylmalonic acid

Ethylmalonic acid is a short-chain organic dicarboxylic acid. Ethylmalonic acid synergistically induces mitochondrial permeability transition (MP) with Ca²⁺, inhibits Mi-CK, and disrupts mitochondrial energy metabolism. Ethylmalonic acid can be used in the research of SCADD, EE and other genetic metabolic diseases characterized by EMA accumulation^[1][2][3][4]. In Vitro:Ethylmalonic acid (0.5-5 mM) dissipates the mitochondrial membrane potential, provokes mitochondrial swelling, impairs mitochondrial Ca²⁺ retention capacity, decreases NAD(P)H matrix content, and does not stimulate hydrogen peroxide generation in isolated brain mitochondria from young rats^[1].
Ethylmalonic acid (0.5-2.5 mM) significantly inhibits mitochondrial creatine kinase (Mi-CK) activity in rat cerebral cortex in a dose-dependent manner, while having no effect on cytosolic creatine kinase (Cy-CK) activity in brain, skeletal muscle, and cardiac muscle^[2].
Ethylmalonic acid (0.5-2.5 mM) increases lipid peroxidation (TBA-RS and chemiluminescence), protein oxidative damage (carbonyl content and sulfhydryl oxidation), DCFH oxidation, and superoxide production, and decreases reduced glutathione (GSH) levels in rat cerebral cortex homogenates^[3].
In Vivo:Ethylmalonic acid (6 μmol/g body weight; s.c.; three doses at 90-min intervals) increases lipid peroxidation (TBA-RS), protein carbonyl content in skeletal muscle, DCFH oxidation and superoxide production in cerebral cortex, decreases GSH levels in skeletal muscle and GPx activity in cerebral cortex in rats^[4].