Fig. 2: PKP2 splice site mutation is sufficient to recapitulate cardiac pathological disease hallmarks associated with ARVC in mice. | Nature Cardiovascular Research

Fig. 2: PKP2 splice site mutation is sufficient to recapitulate cardiac pathological disease hallmarks associated with ARVC in mice.

From: Plakophilin 2 gene therapy prevents and rescues arrhythmogenic right ventricular cardiomyopathy in a mouse model harboring patient genetics

Fig. 2

a,b, Representative cardiac sections stained with hematoxylin and eosin (a) and Masson’s Trichrome staining (b) from control and PKP2 Hom mice at 6 weeks of age. Scale bars, 1 mm. c, High-magnification views of Masson’s Trichrome stained sections from the left ventricle and right ventricle. Scale bars, 50 µm. d, Quantification of fibrosis with RT–qPCR for profibrotic gene markers Col1α1 and Col3α1 (controls, n = 5; Hom, n = 7 biologically independent animals). Data are presented as mean ± s.e.m. Two-tailed unpaired t-test. *P < 0.05 (P = 0.0116 for Col1α1 and P = 0.0448 for Col3α1). e, Right ventricle and left ventricle sections stained with Oil Red O from control and PKP2 Hom hearts at 6 weeks of age. Scale bars, 100 µm. Experiments were repeated independently three times with similar results.

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