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. Author manuscript; available in PMC: 2021 Mar 1.
Published in final edited form as: Clin Chim Acta. 2019 Nov 12;502:233–239. doi: 10.1016/j.cca.2019.11.006

Figure 1. Biosynthesis of organic acids associated with two leucine pathway enzyme deficiencies.

Figure 1.

The leucine catabolism pathway is depicted on the left and right with the position of enzyme deficiencies for HMG CoA lyase (left) and AUH (right) depicted by red filled rectangular boxes. Molecules in the center depict side reactions caused by the buildup of pathway intermediates that ultimately lead to the organic acid products (red boxes) that appear in urine. Numbers refer to specific enzymes (see list). BCAA, branched chain amino acid.