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. 2018 Oct 22;28(4):628–638. doi: 10.1093/hmg/ddy369

Figure 4.

Figure 4

Chemical chaperone activity increases deposition of collagen IV in vivo. (A) Western blotting showed increased levels of Bip (∼2.9-fold increase) and ATF4 (∼4.6-fold increase) in untreated Col4a1+/Svc (SVC unt) which is reduced following PBA treatment. Representative band of total protein stain is given as loading control (the entire gel is provided in Supplementary Material, Fig. S9). (B) ImageJ densitometry analysis of Bip and ATF4 (n = 3). (C) Immunostaining against Col4a1 in PBA-treated and untreated Col4a1+/Svc mice (WT: wild-type; SVC: untreated; SVC 1M PBA: 1-month treatment; SVC LL PBA: lifelong chronic treatment from conception) on kidney sections revealed increased deposition of Col4a1 following PBA treatment. (n = 3–5 mice, Bowman’s capsule: SVC n = 30, SVC 1 M PBA n = 42, SVC LL PBA n = 16; Tubular BM SVC n = 89, SVC 1M PBA n = 77, SVC LL PBA n = 47; Vascular BM SVC n = 36, SVC 1M PBA n = 23, SVC LL PBA n = 15). (D) ImageJ analysis of fluorescence staining. (B), (D) One-way ANOVA post hoc Sidak test *P < 0.05, **P < 0.01, ***P < 0.001.