Rescue of Mffgt phenotypes by deletion of Mfn1. (A) Survival curve demonstrating extension of life with loss of Mfn alleles (n ≥ 14). (B) Echocardiography of different genotypes (n = 3–8). (C) Masson's trichrome stained heart. Bar, 50 µm. (D) Normalized oxygen consumption rates (OCR) of isolated heart mitochondria (≥10 wk; n = 3–5). (E) Respiratory control ratios (n = 3–5). (F) ATP levels in heart (n = 6). (G) Normalized oxidized glutathione levels of 13–14-wk-old hearts (n = 5). (H) Normalized oxidized/reduced glutathione ratio. Increased level in mutants (n = 8) over the wild type (n = 7) is abolished in dm hearts (n = 3). (I and J) Ubiquitin protein blots of 4- (I) and 13–14- (J) wk-old heart mitochondria. Error bars = SEM. P-values reflect comparisons to the wild type unless otherwise noted: ^, P ≤ 0.05; *, P ≤ 0.01; **, P ≤ 0.001. Ant A, complex III inhibitor; CCCP, uncoupler; EF, ejection fraction; Endo, endogenous; FS, fractional shortening; Oligo, ATPase inhibitor; WT, wild type.
