Relative ATPase activities of chABCD3 and mutants in the detergent of LMNG + CHS with 2 mM ATP upon addition of phytanoyl-CoA. The relative activity represents the substrate-stimulated ATPase activity of chABCD3 or its mutants each harboring a single mutation of the conserved residues at the CoA substrate-binding site found in ABCD1. Each data point is the average of three independent experiments, and error bars represent the means ± SD. Student’s t -test was used for the comparison of the phytanoyl-CoA-stimulated ATPase activites between WT chABCD3 and its variants. The P values of R96A, S205A, K209A, Y323A, R373A are 0.0000298, 0.00000464, 0.00000578, 0.00000386 and 0.0000235, respectively. * P < 0.05, ** P < 0.01, *** P < 0.001.