Figure 9. 4-PBA treatment ameliorates aggregate formation in plectin-deficient mice.

(A) Wild-type and MCK-Cre/cKO mice were injected with 4-PBA or vehicle (control) for 10 days. Cryosections of soleus muscles immunolabeled using anti-desmin antibodies are shown. Note reduced formation of desmin aggregates in plectin-deficient muscle after 4-PBA treatment. Scale bar: 20 μm. (B) Teased EDL fibers from MCK-Cre/cKO mice (with or without 4-PBA) were coimmunolabeled for α-actinin and desmin. Scale bar: 20 μm. (C) Immunoblotting of gastrocnemius muscle lysates obtained from mice (with or without 4-PBA).