Chemical Properties
white crystalline powder
Uses
Methylmalonic acid can be used as a substrate in:
- Esterification reactions by various catalysts.
- Synthesis of pyrimidinium betaines with formamidine.
- Synthesis of malonic acid half thioester (MAHT).
Uses
2-Methylpropanedioic acid binds with coenzyme A to form methylmalonyl-CoA, also a metabolic intermediate transformed to succinic acid (SA) by a vitamin B12-dependent catalytic step, and is broadly used as a clinical biomarker of functional vitamin B12 status.
Definition
ChEBI:
Methylmalonic acid is a dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group. It has a role as a human metabolite. It is functionally related to a malonic acid. It is a conjugate acid of a methylmalonate(1-).
Biological Functions
Methylmalonic acid is capable of diffusing out of cells in which it is being produced, so it may be detected in excess in the blood, CSF, and urine of patients with these various forms of the disease.
General Description
Methylmalonic acid is an indicator of vitamin B
12 deficiency and methylmalonic acidemia. This Certified Spiking Solution
? is suitable for use in preparation of calibrators or controls in clinical and diagnostic testing using GC/MS or LC/MS.
Purification Methods
The acid crystallises as the hydrate from water. [Beilstein 2 IV 1932.]
