Fig. 4

Comparison of different ACAD deficiencies in silico. The behaviour of one control and three different ACADD computational models of mFAO are shown. The residual activity reflects typical symptomatic patients: 0% for MCADD and SCADD, and 10% for VLCADD. All other ACADs have 100% of control activity. A Model mitochondrial acyl-CoA profile at 150 µM cytosolic palmitoyl-CoA. B NADH production flux at 150 µM cytosolic palmitoyl-CoA. C Steady-state mitochondrial CoASH. Inset gives the same data with a smaller y-axis