3-Amino-2-methylpropanoic acid

Description: 3-Amino-2-methylpropanoic acid (alpha-Methyl-beta-alanine) is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6), the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities. Target: Endogenous Metabolite. Smiles: CC(CN)C(O)=O. References: Kuilenburg A B P V , Meinsma R , Beke E , et al. beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities.[J]. Human Molecular Genetics, 2004, 13(22):2793-2801.