Fig. 7.

Schematic diagram of interrelationships for selected amino acid abnormalities identified in iMSUD mice in the current study. Note that putrescine (1,4-diaminobutane) and 2-oxoglutarate were not determined in the current study. We speculate that enhanced ammonia production from leucine transamination in brain would disrupt the kinetics of many of the reactions depicted that utilize ammonia in catalysis, in accord with the leucine-glutamate cycle proposed by Yudkoff (1997).