FIGURE 8.

SUMO-defective mutations of VCP impair hexamer assembly. A, pathogenic or SUMO-deficient mutant VCP exerts a comparable inhibitory effect on hexamer formation. HEK293 cells transfected with CFP-SUMO1 together with various VCP constructs were analyzed as described in the legend to Fig. 7A. Values are represented as mean ± S.E. n = 3 experiments. B, reduced VCP hexamer formation in primary kidney epithelial cell cultures from a patient carrying the G97E point mutation in VCP. To minimize the variance among the different clones derived from the initial culturing expansion, 5 clones from the healthy control and 4 from the patient were analyzed for hexamer assembly. The quantification of the data are represented in the adjacent bar graph as mean ± S.E.