2-Methylcitric acid trisodium

[Description]:

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].

[Related Catalog]:

Research Areas >> Metabolic Disease

[Target]

Human Endogenous Metabolite

[In Vitro]

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].

[References]

[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.

[ Molecular Formula ]:
C₇H₇Na₃O₇

[ Molecular Weight ]:
272.10

[ RIDADR ]:
NONH for all modes of transport

The tumour suppressor LKB1 regulates myelination through mitochondrial metabolism.

Nat. Commun. 5 , 4993, (2014)

A prerequisite to myelination of peripheral axons by Schwann cells (SCs) is SC differentiation, and recent evidence indicates that reprogramming from a glycolytic to oxidative metabolism occurs during...

Effect of 2-methylcitrate on citrate metabolism: implications for the management of patients with propionic acidemia and methylmalonic aciduria.

Pediatr. Res. 9 , 905-8, (1975)

2-Methylcitrate was tested in vitro on enzymes which interact with citrate and isocitrate. It was found to inhibit citrate synthase, aconitase, the NAD+- and NADP+-linked isocitrate dehydrogenase. Thi...

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. 185 , 2802-2810, (2003)

The studies reported here identify propionyl coenzyme A (propionyl-CoA) as the common intermediate in the 1,2-propanediol and propionate catabolic pathways of Salmonella enterica serovar Typhimurium L...

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