An endogenous tricarboxylic acid
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2-Methylcitric Acid

Item No. 29327

Technical Information
Formal Name
2-hydroxy-1,2,3-butanetricarboxylic acid
CAS Number
6061-96-7
Molecular Formula
C7H10O7
Formula Weight
Purity
≥95%
A crystalline solid
DMF: 25 mg/mlDMSO: 30 mg/mlEthanol: 10 mg/mlPBS (pH 7.2): 10 mg/ml
SMILES
OC(C(C)C(C(O)=O)(O)CC(O)=O)=O
InChi Code
InChI=1S/C7H10O7/c1-3(5(10)11)7(14,6(12)13)2-4(8)9/h3,14H,2H2,1H3,(H,8,9)(H,10,11)(H,12,13)
InChi Key
YNOXCRMFGMSKIJ-UHFFFAOYSA-N
Shipping & Storage Information
Storage
-20°C
Shipping
Room temperature in continental US; may vary elsewhere
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    Product Description

    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

    WARNING This product is not for human or veterinary use.

    References & Product Citations
    Product Description References

    1. Allen, R.H., Stabler, S.P., Savage, D.G., et alElevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiency. Metabolism 42(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 acidemias. J. Med. Screen. 24(2), 58-61 (2017).