2-Hydroxy-3-methylbutyric acid

• 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.

• Stability:

  ≥ 2 years

• Purity:

  >98% (HPLC)

• Weight:

  118.13

• Molecular Formula:

  C5H10O3

• Notes:

  Research use only, not for human use.

• Receptor:

  Others

• CAS Number:

  4026-18-0