Figure 2

Specific enzyme complex activity of acyl-CoA dehydrogenase deficiencies. (A) Activity distribution of four distinct acyl-CoA dehydrogenases with respect to fatty acid carbon chain length based on data from literature [3–5]. Short-chain (SCAD), medium-chain (MCAD), long-chain (LCAD) and very long-chain (VLCAD) acyl-CoA dehydrogenase is defined according to the specific enzyme complex activity catalyzing dehydration of short-, medium-, long- and very long-chain fatty acids. The total activity of all four acyl-CoA dehydrogenases is shown in red (Total). (B) Total acyl-CoA dehydrogenase activity as a function of carbon chain length without deficiencies in enzyme activity (Control). Reduced activity of acyl-CoA dehydrogenases yields to different total activity reflecting deficiencies of short-chain (SCADD), medium-chain (MCADD), long-chain (LCADD) and very long-chain (VLCADD) acyl-CoA dehydrogenase, respectively.