Fig. 7
Steady-state mFAO flux and mitochondrial CoASH in personalised patient models. Fibroblasts proteomics from four phenotypic groups were used to personalise the kinetic model of mFAO: Controls (n = 5), symptomatic (n = 4), early diagnosis, (ED, n = 5) and asymptomatic (n = 1). All simulations were done at 150 µM cytosolic palmitoyl-CoA. A Normalised, measured expression of key β-oxidation proteins per clinical group. Individual people are averages of at least three technical replicates and are represented as data points in the distribution bars. HADHa and HADHb are the two subunits of MTP. B, C Simulation results from personalised computational models: flux and mitochondrial CoASH concentration. Dashed lines indicate the region of the Km,CoASH values of key mitochondrial enzymes: PDH, αKDH, MCKAT and CrAT (Table 2). Control and MCADD were compared using a t-test