An endogenous tricarboxylic acid
2-Methylcitric acid is an endogenous tricarboxylic acid formed by the condensation of propionyl-CoA with oxaloacetic acid by citrate synthase under conditions of propionyl-CoA accumulation.1 Accumulation of 2-methylcitric acid is associated with cobalamin deficiencies, propionic acidemia, and methylmalonic acidurias.1,2
1.Allen, R.H., Stabler, S.P., Savage, D.G., et al.Elevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiencyMetabolism42(8)978-988(1993) 2.Al-Dirbashi, O.Y., McIntosh, N., and Chakraborty, P.Quantification of 2-methylcitric acid in dried blood spots improves newborn screening for propionic and methylmalonic acidemiasJ. Med. Screen.24(2)58-61(2017)
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