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Case Reports
. 1971 Dec 11;2(7737):1289-90.
doi: 10.1016/s0140-6736(71)90605-2.

A "new" disorder of isoleucine catabolism

R S Daum, P H Lamm, O A Mamer, C R Scriver
Case Reports

A "new" disorder of isoleucine catabolism

R S Daum et al. Lancet. .

Abstract

A disorder of isoleucine catabolism was found in a child with intermittent metabolic acidosis. The block occurs at the stage of propionate synthesis, and two intermediates--alpha-methylacetoacetate and alpha-methyl-beta-hydroxybutyrate--accumulate. The condition is apparently inherited.

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