Fig. 5.
The V190 mutant can function in mitochondria. Wild type and mutant ALAS-E proteins were expressed in QT6 cells. Panel A shows cell homogenate under normal lamp. The porphyrin accumulation in the cells was measured by exposing the cell homogenate (B) and culture supernatant (C) to UV light. Tube 1 contains cells transfected with control plasmid. Cell homogenate and culture supernatant expressing wild type ALAS-E (tube 2) displays an intense red fluorescence under UV lamp, indicating the presence of porphyrins. Expression of the V190 mutant (tube 3) also gave rise to red fluorescence, albeit of weaker intensity than wild type, while cell homogenate and culture supernatant transfected with the V426 mutant plasmid (tube 4) showed only marginal red fluorescence.