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2-Methylcitric acid (Methylcitric acid) is a metabolic marker of methylmalonic acidemia that induces mitochondrial permeability transition (PT) and reduces ATP production in brain mitochondria.
Description | 2-Methylcitric acid (Methylcitric acid) is a metabolic marker of methylmalonic acidemia that induces mitochondrial permeability transition (PT) and reduces ATP production in brain mitochondria. |
In vitro | 2-Methylcitric acid has a significant effect on glutamate metabolism and interferes with mitochondrial function through a variety of mechanisms. It inhibits glutamate dehydrogenase (GDH) activity at concentrations as low as 0.5 mM, which in turn affects glutamate oxidation. In addition, 2-Methylcitric acid acts on mitochondria at concentrations of 1-3 mM, causing a significant decrease in membrane potential and mitochondrial swelling, a process that is caused by its ability to inhibit calcium loading supported by different substrates.2-Methylcitric acid has been shown to interfere with mitochondrial function by inhibiting glutamate dehydrogenase (GDH) activity at concentrations as low as 0.5 mM. The compound disrupts mitochondrial energy homeostasis by inhibiting glutamate dehydrogenase activity and acting as an inducer of permeability transition. Notably, although 2-Methylcitric acid disrupts mitochondrial energy homeostasis, it does not significantly affect glutamate transport function in liver mitochondria. [1] |
Alias | Methylcitric acid |
Molecular Weight | 206.15 |
Formula | C7H10O7 |
Cas No. | 6061-96-7 |
Smiles | O=C(O)CC(O)(C(=O)O)C(C(=O)O)C |
Relative Density. | 1.178g/cm3 |
Storage | store at low temperature | Powder: -20°C for 3 years | In solvent: -80°C for 1 year | Shipping with blue ice. | |||||||||||||||||||||||||||||||||||
Solubility Information | DMSO: 80 mg/mL (388.07 mM), Sonication is recommended. H2O: 80 mg/mL (388.07 mM), Sonication is recommended. | |||||||||||||||||||||||||||||||||||
Solution Preparation Table | ||||||||||||||||||||||||||||||||||||
DMSO/H2O
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