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Fig. 4 | BMC Biology

Fig. 4

From: Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders

Fig. 4

Experimental and simulated acylcarnitine profiles of two MADD patients, differing in disease severity, and the consequences for the simulated mFAO flux in those patients. Panels a and b: Experimental data for plasma concentrations from a healthy population (red bars; data represents mean ± standard deviation (n = 1750) and the two MADD patients (single time point; blue bars; panel a: patient 1; panel b: patient 2). Note that the same control data were used for a and b, but due to the different Y-axis scales, they are barely visible in panel b. Panels c and d: Simulated acylcarnitine profiles for healthy subjects (red bars; FADH2 concentration of 0.43 μM), patient 1 (blue bars panel c; FADH2 concentration 0.6 μM) and patient 2 (blue bars panel d; FADH2 concentration 0.73 μM). Simulations were performed at a constant sum of FADH2 and FAD of 0.77 μM. The dark blue and red bars are the concentrations simulated in the human model with competition and the light blue and red bars are the concentrations simulated in the human model without competition. Panel e: Steady-state rates of flux at increasing concentrations of the substrate palmitoyl-CoA for healthy subjects (solid red line; FADH2 concentration of 0.43 μM), patient 1 (dotted blue line; FADH2 concentration 0.6 μM) and patient 2 (dashed blue line; FADH2 concentration 0.73 μM) simulated in the model with competition. Panel f: Steady-state rates of flux at increasing concentrations of the substrate palmitoyl-CoA for healthy subjects (solid red line; FADH2 concentration of 0.43 μM), patient 1 (dotted blue line; FADH2 concentration 0.6 μM) and patient 2 (dashed blue line; FADH2 concentration 0.73 μM) simulated in the model without competition

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