Abstract
At present six patients with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, an inborn error of leucine metabolism have been reported [l-4 and C. Bachmann, personal communication]. Characteristic urinary organic acids in this disease are: 3-hydroxy-3-methylglutaric acid, 3-methylglutaconic acid, 3-methylglutaric acid and 3-hydroxyisovaleric acid. Endogenous accumulation of 3-methylcrotonyl-CoA leads to the formation of 3-methylcrotonylglycine [ 51. However, urinary 3-methylcrotonic acid may also be formed artificially by decarboxylation of 3-methylglutaconic acid [ 61. During the investigation of the decarboxylation of 3-methylglutaconic acid another compound was detected, probably an isomer of 3-methylcrotonic acid. In this article the identification of this compound as 3-methyl-3-butenoic acid is described. Evidence is presented that 3-methyl-3-butenoic acid is not an endogenous metabolite.
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