Aminomalonic acid (Ama) was first detected in alkaline hydrolysates of proteins in 1984. Ama has been isolated from proteins of Escherichia coli and human atherosclerotic plaque. The presence of Ama has important biological implications because the malonic acid moiety potentially imparts calcium binding properties to protein.

2-Hydroxy-2-methylbutyric acid is an unusual metabolite found in the urine of patients with 2-hydroxyglutaric aciduria and maple syrup urine disease.

KV4 channel blocker. Blocks A-type K+ current (ISA) in mouse cerebellar granule neurons. The accessory dipeptidyl peptidase-like proteins (DPP) 6 and 10 are required for blockade.