2-Methylcitric acid trisodium (Synonyms: Methylcitric acid trisodium)

2-Methylcitric acid trisodium (Methylcitric acid trisodium) is an endogenous metabolite in the 2-methylcitric acid cycle. 2-Methylcitric acid trisodium accumulates in methylmalonic and propionic acidemias and acts as a marker metabolite. 2-Methylcitric acid trisodium markedly inhibits ADP-stimulated and uncoupled respiration in mitochondria supported by glutamate[1].

2-Methylcitric acid trisodium impairs glutamate metabolism and induces permeability transition in brain mitochondria[1].2-Methylcitric acid trisodium inhibits glutamate dehydrogenase (GDH) activity at concentrations as low as 0.5 mM[1].2-Methylcitric acid trisodium (1 mM, 3 mM; 150 seconds) provokes a significant decrease in mitochondrial membrane potential and induced swelling in Ca2+-loaded mitochondria supported by different substrates[1].2-Methylcitric acid trisodium behaves as a potent inhibitor of glutamate oxidation by inhibiting glutamate dehydrogenase activity and as a permeability transition inducer, disturbing mitochondrial energy homeostasis[1].2-Methylcitric acid trisodium does not significantly impair mitochondrial glutamate transport[1].2-Methylcitric acid trisodium does not significantly impair glutamate mitochondrial transport in liver mitochondria[1].

[1]. Palacios S, et al. Propionyl Coenzyme A Is a Common Intermediate in the 1,2-Propanediol and Propionate Catabolic Pathways Needed for Expression of the prpBCDE Operon during Growth of Salmonella enterica on 1,2-Propanediol. J Bacteriol. 2003 May;185(9):2802-10.