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An isotope labelled derivative of Methylmalonic Acid (M318860), 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.

Methyl-d3-malonic acid is an isotope labelled derivative of Methylmalonic Acid (M318860), binds with coenzyme A to form methylmalonyl-CoA, also a metabolic intermediate transformed to succinic acid (SA) by a vitamin B12-depend ent catalytic step, and is broadly used as a clinical biomarker of functional vitamin B12 status.

Methylmalonic acid, a malonic acid derivative, is a vital intermediate in the metabolism of fat and proteins and plays a key role in energy metabolism. Elevated methylmalonic acid levels can be caused by an inborn error of metabolism leading to methylmalonic aciduria or indicate early vitamin B₁₂ deficiency. This internal standard is suitable for quantitation of methylmalonic acid levels by LC/MS or GC/MS in newborn screening and clinical testing applications to test for methylmalonic acidemia and diagnose vitamin B₁₂ deficiency.