Figure 1
From: Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency
Long-chain fatty acid β-oxidation (LC-FAO) flux, very-long-chain acyl-CoA dehydrogenase (VLCAD) activity, and acylcarnitine assay. (a) LC-FAO flux ([9,10-3H(N)]-oleic acid oxidation rate) and (b) VLCAD activity (with palmitoyl-CoA as substrate) in cells from 13 patients were measured after being cultured for 2 weeks at 30, 37, and 40 °C. Concentrations of various (long-chain) acylcarnitines after 96 hours of incubation with [U-13C]palmitate at 37 °C were plotted, including (c) C12,C14,C16, (d) C2, (e) and their corresponding ratios: C12/C16, C14/C16, and C14/C2. Patient identification numbers (PIDs) were assigned based on the results of LC-FAO flux analysis at 37 °C. PID 1 had the lowest and PID 13 the highest LC-FAO flux.
