Figure 4. Product and intermediate profiles of the URO-D reaction.
Reaction products of either uroporphyrinogen-I (A) or -III (B) from the URO-D enzymatic assay are separated by HPLC, the metabolites heptacarboxyl porphyrin (7), hexacarboxyl porphyrin (6), pentacarboxyl porphyrin (5) and coproporphyrin (4) are labeled with the corresponding isomer designation after the label. WT enzyme (pink), scURO-D (purple), scURO-D(FY) (green), scURO-D(YF) (blue) and scURO-D(FF) (red).