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Case Reports
. 1976 Aug 2;70(3):399-406.
doi: 10.1016/0009-8981(76)90353-3.

3-Hydroxy-3-methylglutaric aciduria, combined with 3-methylglutaconic aciduria

S J Wysocki, S P Wilkinson, R Hähnel, C Y Wong, P K Panegyres
Case Reports

3-Hydroxy-3-methylglutaric aciduria, combined with 3-methylglutaconic aciduria

S J Wysocki et al. Clin Chim Acta. .

Abstract

1. A baby with severe metabolic acidosis was found to excrete abnormal amounts of 3-methylcrotonic acid, 3-methylglutaconic acid and 3-hydroxy-3-methylglutaric acid in urine. 2. Several other abnormal constitutents appear to be products of side-reactions and include 3-hydroxy-3-methylbutyric acid and 3-methylglutaric acid. 3. The profile of acids in urine indicates a blockage in the sixth step of leucine catabolism, the cleavage of 3-hydroxy-3-methylglutaryl-CoA to acetoacetic acid and acetyl-CoA.

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