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

Figure 8. Base editing to correct DSP p.R1951X reduces inflammation in EHTs.

Figure 8

(A) Sanger sequencing of p.R1951X hiPSCs before and after base editing to correct the pathogenic DSP variant using gRNA8 and an adenine base editor (ABE). Base editing restored the reading frame, and the corrected line was termed p.R1951XCor. (B) Fractional shortening (FS) measurements of p.R1951X and p.R1951XCor EHTs showed improved contractility following genomic correction (****< 0.0001 by 2-tailed t test with Welch’s correction; n = 15 per condition). (C and D) Cytokine arrays of EHT media demonstrate significant reduction of cytokine secretion following genomic correction of DSP p.R1951X. Targets displayed in colored boxes are quantified in relation to Reference Dots (D) (†< 0.0001, ‡< 0.001, ◊< 0.05 by 2-way ANOVA with n = 4 per condition). Data presented as mean ± SEM.