title: Accumulation of 3-hydroxyisobutyric acid, 2-methyl-3-hydroxybutyric acid and 3-hydroxyisovaleric acid in ketoacidosis.
abstract: 1. Urine and serum samples from patients with ketoacidosis of varying degree and etiology have been examined by gas chromatography and mass spectrometry. 2. In addition to 3-hydroxyisovaleric acid, relatively high concentrations of two analogous hydroxy acids, 3-hydroxyisobutyric acid and 2-methyl-3-hydroxybutyric acid, were found in the urine. 3. There were highly significant positive correlations between the excreted amounts of the three acids. 4. Experiments on rats with isotope-labelled compounds revealed that the acids were formed by the degradation of leucine, isoleucine and valine. 5. The accumulation of the hydroxy acids during ketoacidosis is probably caused by a similar derangement of the metabolism of all three branched-chain amino acids.
mesh_terms: D000085:Acetates / Q000378:metabolism; D000138:Acidosis / Q000378:metabolism*; D000818:Animals; D002648:Child; D002675:Child, Preschool; D002849:Chromatography, Gas; D003920:Diabetes Mellitus / Q000139:chemically induced; D016883:Diabetic Ketoacidosis / Q000378:metabolism; D005977:Glutarates / Q000378:metabolism; D006801:Humans; D006880:Hydroxy Acids / Q000378:metabolism*; D006885:Hydroxybutyrates / Q000378:metabolism*; D007223:Infant; D007532:Isoleucine / Q000378:metabolism; D007930:Leucine / Q000378:metabolism; D008297:Male; D013058:Mass Spectrometry; D051381:Rats; D013311:Streptozocin; D014631:Valerates / Q000378:metabolism*; D014633:Valine / Q000378:metabolism
chemical_list: D000085:Acetates; D005977:Glutarates; D006880:Hydroxy Acids; D006885:Hydroxybutyrates; D014631:Valerates; D007532:Isoleucine; D013311:Streptozocin; D007930:Leucine; D014633:Valine