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. 2015 May 27;24(1):142–145. doi: 10.1038/ejhg.2015.106

Figure 2.

Figure 2

Visualization of the valine degradation pathway and the interaction with LDH. Abbreviations of enzyme names are shown in boldface BCAT: branched-chain aminotransferase; BCKDH: branched-chain alpha-keto acid dehydrogenase; LDH: lactate dehydrogenase. LDH can convert 3-methyl-2-oxobutanoate, which is the product of valine after transamination, into alpha-hydroxyisovalerate to balance the redox potential under hypoxic conditions. Under aerobic conditions, 3-methyl-2-oxobutanoate can be further degraded by the enzymes of branched-chain amino acid catabolism. In contrast, alpha-hydroxyisovalerate cannot be metabolized any further and is excreted in urine.