L-Alloisoleucine

L-Alloisoleucine is a branched-chain amino acid and diastereomer of L-isoleucine. It is formed from L-isoleucine by transamination. Plasma L-alloisoleucine levels are increased in patients with maple syrup urine disease (MSUD), an inborn error of metabolism characterized by a deficiency in the branched-chain alpha-ketoacid dehydrogenase complex, the complex that catalyzes the degradation of branched-chain amino acids, leading to the accumulation of branched-chain amino acids in the plasma and urine, a maple syrup-like odor in the urine, and neurological impairments.CAS Number: 1509-34-8. Synonyms: NSC 206282. Molecular Formula: C6H13NO2. Formula Weight: 131.2. Purity: >95%. Formulation: (Request formulation change), A solid. Solubility: DMF: insol, DMSO: insol, Ethanol: insol, PBS (pH 7.2): 1 mg/ml. SMILES: O=C([C@H]([C@@H](CC)C)N)O. InChi Code: InChI=1S/C6H13NO2/c1-3-4(2)5(7)6(8)9/h4-5H,3,7H2,1-2H3,(H,8,9)/t4-,5+/m1/s1. InChi Key: AGPKZVBTJJNPAG-UHNVWZDZSA-N.