isovaleric acid /-vəler″ik/ [Gk, isos + L, valeriana, herb, acidus, sour] , a fatty acid with a pungent taste and disagreeable odor that is found in valerian and other plant products, as well as in cheese. It also occurs as a metabolite of the amino acid leucine and is found in the sweat of feet and in the urine of patients with smallpox, hepatitis, and typhus. It has been used commercially in a variety of drugs, perfumes, and flavorings. Isovaleric acidemia occurs in patients who have abnormally high levels of isovaleric acid in the blood and urine as a result of an inherited deficiency of the enzyme isovaleryl coenzyme A dehydrogenase. The condition is treated with diets that contain low-leucine foods. Also called isopentoic acid, isopropylacetic acid.
