Figure 8.

Enhanced dynein-mediated trafficking and degradation of nephrin in PAN model in R218Q KI mice. (A) Dynll1 and ubiquitinated nephrin were detected in the nephrin pulldown of kidney lysates by immunoblotting, and biotinylated nephrin was detected in streptavidin pulldown. Semiquantitative analysis was performed to compare Dynll1/total nephrin pulldown, biotinylated nephrin/total nephrin, ubiquitinated nephrin/total nephrin in mice (wt/wt, wt/ki and ki/ki), with PA or without PA injection using an independent sample t test (n=5). P<0.05 PAN versus control mice of the same genotype without PA injection. (B) Immunofluorescent stain showed increased colocalization of Dynll1 with nephrin in PAN of R218 KI mice (wt/ki and ki/ki) compared with the wt/wt mice. (C) Increased Dynll1 and decreased nephrin expression at the GBM of sclerosing glomeruli of the wt/ki and ki/ki mice with PAN.