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. 2014 Feb 3;124(3):1144–1157. doi: 10.1172/JCI71919

Figure 7. Treatment of differentiated Plec+/+ and Plec–/– myotubes with the chemical chaperone 4-PBA.

Figure 7

(A) 4-PBA–treated (1 mM) Plec+/+ and Plec–/– myotubes (differentiated for 10 days) were scored for cells with aggregated IFs. Mean ± SEM, 3 experiments (Plec+/+, n = 358; Plec–/–, n = 376). Note drastic reduction of affected myotubes in Plec–/– cells compared with untreated cells (dashed lines). (B) Dose response block diagram displaying the statistical evaluation of desmin protein aggregate formation in Plec–/– myotubes differentiated for 10 days in the presence of increasing concentrations of 4-PBA. (C) Z-disk formation in Plec+/+ and Plec–/– myotubes differentiated as in A. Mean ± SEM, 3 experiments (Plec+/+, n = 357; Plec–/–, n = 438). Note marked stabilization of sarcomeres in Plec–/– myotubes compared to untreated cells (dashed lines). (D) Dose response block diagram showing the statistical evaluation of Plec–/– cells with intact Z-disk formation after differentiation for 10 days in the presence of increasing concentrations of 4-PBA. Data in B and D represent mean ± SEM, 3 experiments. (E) Immunoblotting of cell lysates from 4-PBA–treated Plec+/+ and Plec–/– myotubes using antibodies to proteins indicated.

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