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. 2024 May 16;134(13):e180254. doi: 10.1172/JCI180254

Figure 6. Improved contractile function with NF-κB inhibition in DSP EHTs.

Figure 6

(A) Treatment with BAY 11-7082, an NF-κB inhibitor, improved fractional shortening of DSP p.E1597X and p.R1951X EHTs with no significant effect on healthy control cells (*< 0.05, **< 0.01 by 2-way ANOVA, n = 9–12 EHTs per condition, data representative of 3 batches). (B and C) BAY 11-7082 improved strain-induced force loss in DSP p.E1597X EHTs as measured in force time integral (B) and active force (C) (*< 0.05, **< 0.01 by 2-way ANOVA, n = 3–4 EHTs per condition across 2 batches, labeled as n1 = black dots, n2 = white dots, n3 = gray dots, n4 = brown dots). Box plots show the interquartile range, median (line), and minimum and maximum (whiskers). (D and E) Cytokine arrays of p.E1597X EHT media showed a significant reduction of baseline cytokine secretion following BAY 11-7082 treatment (†< 0.0001, ‡< 0.001, Δ< 0.01, ◊< 0.05 by 2-way ANOVA with n = 4 per condition). Data presented as individual recordings normalized to average baseline measurement per EHT. Significance calculated based on mean value per EHT.