Isovaleroylglycine

Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase. It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid beta-oxidation defects.Formal Name: N-(3-methyl-1-oxobutyl)-glycine. CAS Number: 16284-60-9. Synonyms: N-Isovalerylglycine. Molecular Formula: C7H13NO3. Formula Weight: 159.2. Purity: >95%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMSO: 5 mg/ml, Methanol: 1 mg/ml. SMILES: OC(CNC(CC(C)C)=O)=O. InChi Code: InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11). InChi Key: ZRQXMKMBBMNNQC-UHFFFAOYSA-N.