2-Methylcitric acid trisodium (Methylcitric acid trisodium) 是 2- 甲基柠檬酸循环的内源性代谢物。2-Methylcitric acid trisodium 在甲基丙二酸血症和丙二酸酸血症中积累并能作为代谢标志物。2-Methylcitric acid trisodium 对谷氨酸支持的线粒体中 ADP 刺激和不耦合呼吸作用有明显的抑制作用。

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.

Solid

Human Endogenous Metabolite

2-Methylcitric acid trisodium impairs glutamate metabolism and induces permeability transition in brain mitochondria.
2-Methylcitric acid trisodium inhibits glutamate dehydrogenase (GDH) activity at concentrations as low as 0.5 mM.
2-Methylcitric acid trisodium (1 mM, 3 mM; 150 seconds) provokes a significant decrease in mitochondrial membrane potential and induced swelling in Ca-loaded mitochondria supported by different substrates.
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.
2-Methylcitric acid trisodium does not significantly impair mitochondrial glutamate transport.
2-Methylcitric acid trisodium does not significantly impair glutamate mitochondrial transport in liver mitochondria.

Room temperature in continental US; may vary elsewhere.

4°C, sealed storage, away from moistur In solvent : -80°C, 6 months; -20°C, 1 month (sealed storage, away from moisture)

[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):280