Ariadne Genomics Inc. NADH Hadha 3-ketoacyl-CoA Acaa2 3-hydroxyacyl-CoA Hadh Echs1 FADH2 CoQ e2 e1 Etfdh Etfb Cpt2 Slc25a20 Acs Cpt1 Acad CoQ ---> oxidative phosphorylation pathway carnitine-in carnitine-out acylcarnitine translocated fatty acyl-CoA Hadhb NAD ---> NADH NAD translocated fatty acyl-CoA ---> trans-2-enoyl-CoA fatty acids ---> fatty acyl-CoA 3-ketoacyl-CoA ---> acyl-CoA - 2C 3-hydroxyacyl-CoA <--> trans-2-enoyl-CoA 3-hydroxyacyl-CoA <--> 3-ketoacyl-CoA trans-2-enoyl-CoA fatty acids fatty acyl-CoA ---> acylcarnitine carnitine-out ---> acylcarnitine carnitine-in ---> carnitine-out acylcarnitine ---> carnitine-in acylcarnitine ---> translocated fatty acyl-CoA FADH2 ---> e1 FADH2 ---> e2 e1 ---> CoQ e2 ---> CoQ acetyl-CoA ---> citrate cycle pathway acetyl-CoA ---> ketone bodies metabolic pathway acyl-CoA - 2C ---> translocated fatty acyl-CoA 3-ketoacyl-CoA ---> acetyl-CoA FAD FAD ---> FADH2 fatty acyl-CoA acetyl-CoA acylcarnitine ---> translocated fatty acyl-CoA citrate cycle pathway ketone bodies metabolic pathway acyl-CoA - 2C Etfa oxidative phosphorylation pathway