25-Dimethylpyrazine is present in asparagus wheat bread crispbread Swiss cheeses black or green tea soya malt raw shrimp and squid. 25-Dimethylpyrazine is used as flavor additive and odorant in foods.

Protein Kinase A Substrate

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.