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2-Hydroxy-3-methylbutyric acid

CAS No. 4026-18-0

2-Hydroxy-3-methylbutyric acid( 2-Hydroxy-3-methylbutanoic acid | (±)-2-Hydroxyisopentanoic Acid | 2-Hydroxyisovaleric Acid )

Catalog No. M19995 CAS No. 4026-18-0

2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.

Purity : >98% (HPLC)

COA Datasheet HNMR HPLC MSDS Handing Instructions
Size Price / USD Stock Quantity
1 mL x 10 mM in DMSO 28 In Stock
100MG 29 In Stock
200MG Get Quote In Stock
500MG 65 In Stock
1G 91 In Stock
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Biological Information

  • Product Name
    2-Hydroxy-3-methylbutyric acid
  • Note
    Research use only, not for human use.
  • Brief Description
    2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.
  • Description
    2-Hydroxy-3-methylbutyric acid (2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria methylmalonic acidemia propionic acidemia 3-ketothiolase deficiency isovaleric acidemia 3-methylcrotonylglycemia 3-hydroxy-3-methylglutaric acidemia multiple carboxylase deficiency glutaric aciduria ornithine transcarbamylase deficiency glyceroluria tyrosinemia type I galactosemia and maple syrup urine disease.
  • In Vitro
    ——
  • In Vivo
    ——
  • Synonyms
    2-Hydroxy-3-methylbutanoic acid | (±)-2-Hydroxyisopentanoic Acid | 2-Hydroxyisovaleric Acid
  • Pathway
    Others
  • Target
    Other Targets
  • Recptor
    Others
  • Research Area
    ——
  • Indication
    ——

Chemical Information

  • CAS Number
    4026-18-0
  • Formula Weight
    118.13
  • Molecular Formula
    C5H10O3
  • Purity
    >98% (HPLC)
  • Solubility
    DMSO:194.7 mM
  • SMILES
    CC(C)C(O)C(O)=O
  • Chemical Name
    ——

Shipping & Storage Information

  • Storage
    (-20℃)
  • Shipping
    With Ice Pack
  • Stability
    ≥ 2 years

Reference

1.Magni F et al. Simultaneous determination of plasma levels of alpha-ketoisocaproic acid and leucine and evaluation of alpha-[1-13C]ketoisocaproic acid and [1-13C]leucine enrichment by gas chromatography-mass spectrometry. Anal Biochem. 1994 Aug 1;220(2):308-14.
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