Name: Ketoisovaleric acid
Price: 180.00 USD

- Overview
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    Background
    
    Ketoisovaleric acid is an abnormal metabolite originated from the incomplete breakdown of branched-chain amino acids. Chronically high levels of ketoisovaleric acid are associated with maple syrup urine disease (MSUD). MSUD is an inherited metabolic disorder caused by deficiency of branched-chain α-keto acid dehydrogenase complex activity which leads to accumulation of the branched-chain α-keto acids including α-ketoisocaproic acid, α-ketoisovaleric acid and α-keto-β-methylvaleric acid, as well as their respective amino acids in blood and urine. From a chemistry perspective, ketoisovaleric acid is a keto acid, which is a subclass of organic acids. Abnormally high levels of organic acids in blood (organic acidemia), urine (organic aciduria), the brain, as well as other tissues result in general metabolic acidosis.
    
    1. HMDB0000019 (alpha-Ketoisovaleric acid). The Human Metabolome Database. Retrieved on September 7, 2021.
    2. Bridi R, Braun CA, Zorzi GK, et al. alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats. Metabolic Brain Disease, 2005, 20(2): 155-167.
- Properties
  - Categories
    
    An abnormal metabolite originated from the incomplete breakdown of branched-chain amino acids
    
    Alternative Name
    
    3-methyl-2-oxobutanoic acid
    
    CAS Number
    
    759-05-7
    
    Molecular Formula
    
    C5H8O3
    
    Molecular Weight
    
    116.12
    
    Appearance
    
    A colorless liquid
    
    Purity
    
    98.00%
    
    Solubility
    
    ≥11.6 mg/mL in DMSO; ≥27.05 mg/mL in H2O; ≥76.5 mg/mL in EtOH
    
    Storage
    
    Store at -20°C
    
    SMILES
    
    CC(C(C(O)=O)=O)C
    
    * For Research Use Only