Electron microscopy localization of procollagen-I in fibroblasts of carriers of the FFI mutation.A and B, immunogold staining on cryosections from FFI human fibroblasts using anti-PC-I, showing PC-I accumulation in dilated ER cisternae and in the Golgi. C, double immunogold staining using anti-PC-I (5 nm gold particles) and anti-GM130 antibodies (10 nm gold particles), showing accumulation of PC-I in the trans- but not in the cis- (GM130-positive) cisternae of the Golgi complex. Scale bars 500 nm. D, quantification of gold particles in different cell compartments. Bars indicate the mean ± SD of 5 to 26 images. ∗p < 0.05; ∗∗∗∗p < 0.0001 versus WT by two-way ANOVA Bonferroni’s post-hoc test.