A simple method of determining 4-hydroxyisovaleric acid and its level in a patient with isovaleric acidemia.
We developed a simple and rapid method for the quantitative determination of 4- hydroxyisovaleric acid using the conversion of this acid to the corresponding lactone,and measured its levels in the plasma of a patient with isovaleric acidemia during an episode of severe ketoacidosis. 4-Hydroxyisovaleric acid reached a peak two days after plasma isovaleric acid was at its highest level.
D000138:Acidosis / Q000097:blood*; D008401:Gas Chromatography-Mass Spectrometry; D045782:Hemiterpenes; D006801:Humans; D007662:Ketosis / Q000097:blood* / Q000652:urine; D010421:Pentanoic Acids / Q000097:blood*; D014631:Valerates / Q000097:blood* / Q000652:urine
D045782:Hemiterpenes; D010421:Pentanoic Acids; D014631:Valerates; C008216:isovaleric acid; C004961:beta-hydroxyisovaleric acid; C030032:4-hydroxyisovaleric acid