Fig. 3: Administration of AAV9-hNdufs6 prevented pathological changes in newborn Ndufs6gt/gt mice.
From: Gene therapy prevents onset of mitochondrial cardiomyopathy in neonatal mice with Ndufs6 deficiency
A Representative images illustrating the morphology of heart. Scale bar = 1 mm. B Quantitative analysis of LVEDD of WT, Ndufs6gt/gt and AAV-Neonatal mice at 3 months old (n = 6). C Quantitative analysis of HW/BW ratio of WT, Ndufs6gt/gt and AAV-Neonatal mice at 3-months old (n = 6). D Representative images of Masson’s trichrome staining and quantitative measurement of heart fibrosis (n = 4). Scale bar = 100 μm. E Representative images of TUNEL staining and quantification analysis of the heart tissue from WT, Ndufs6gt/gt and AAV-Neonatal mice (n = 4). Scale bar = 50 μm. The tissue slides utilized are consecutive sections from the same specimen stained with Masson’s trichrome on (D). F Representative mitochondrial ultrastructure and quantitative measurement of mitochondrial fragmentation in different experimental groups (n = 4). Scale bar = 1 μm. G Representative images of mitochondrial cristae morphology and the quantification of the degree of cristae damage, scale bar = 500 nm in WT, Ndufs6gt/gt and AAV-Neonatal groups. The results were calculated based on mitochondrial classification system (n = 4, with at least 100 mitochondria averaged for each replicate).
