Skip to main content
. 2011 Sep 14;6(9):e24738. doi: 10.1371/journal.pone.0024738

Figure 6. Pharmacological rescue of ABCC6 mutants.

Figure 6

A: Mice were treated with 4-PBA prior to being subjected to HTVI with plasmids expressing the WT ABCC6 and mutants R1339C, R1138Q and R1314W. Immunostaining on frozen sections of mouse liver were performed to reveal both the mouse Abcc6 (red) and the human ABCC6 (green). The arrows show the improved plasma membrane localization of mutant R1314W as compared to the untreated controls. Each image was derived from a different mouse. The scale bar represents 50 µm. B: Co-localization between the endogenous ER marker Calnexin and mutant R1314W is indicated by yellow color. Treatment with 4-PBA facilitated the release of the latter mutant from the ER as indicated by the reduced co-localization. C: Effect of 1 mM 4-PBA treatment on the intracellular localization of R1314W in MDCKII cells as detected by immunofluorescence using the M6II-7 monoclonal antibody. The improved cellular localization of mutant R1314W is indicated by arrows.