Figure 3 .

Enzyme activities after expression of wild-type and mutant SBCAD and ACAD-8 in COS-7 cells. The results from measuring ACAD activity in COS-7 cells transfected with pSBCAD-WT, pSBCAD-MUT, or pACAD-8-WT expression vectors are shown. The control is COS-7 cells harboring the expression vector pcDNA3.1+ with no cDNA insert (Control). Activity measurements are given in nmol/min/mg protein. Blackened bars show the enzyme activity measured with 2-methylbutyryl-CoA as substrate, and gray bars show the enzyme activity measured with isobutyryl-CoA as substrate. Error bars indicate the range of the values measured. None of the transfected cells showed activity above background when either butyryl-CoA or isovaleryl-CoA was used as substrate (not shown).