Fig. 6.

Decrease in PSMD13-mediated degradation and inclusion body formation of the mutant RPE65s at low temperature. (A) ARPE-19 cells co-transfected with RPE65 and pPSMD13 or pRK5 were grown at 37 or 30 °C and subjected to immunoblot analysis using antibodies against RPE65 or β-actin. Histograms show relative expression levels of WT and the mutant RPE65 proteins in the pPSMD13- or the mock vector-co-transfected cells grown at 37 or 30 °C. (B) Representative confocal microscopic images of ARPE-19 cells transfected with WT or mutant RPE65 construct. The cells were incubated at 37 or 30 °C, and stained with a RPE65 antibody. Scale bars denote 10 µm.