Lack of Correlation between Outcomes of Membrane Repair Assay and Correction of Dystrophic Changes in Experimental Therapeutic Strategy in Dysferlinopathy
Figure 1
Characterization of the Myoferlin transgenic mice. A
/Schematic representation of the Myof construct and Hprt targeted allele. B/Quantitative RT-PCR for myoferlin expression in muscles of WT and TgMyof revealed a 200-fold greater expression in the transgenic. The data are presented relative to a titration with the original myoferlin expression plasmid. (** p<0.01 between WT and TgMyof). C/Western blot for the myoferlin protein in WT and TgMyof showing the overexpression in the transgenic animals (results for 2 animals at 3 weeks and 4 months of age are shown). A-actinin staining was used to confirm that similar amounts of total protein extracted from muscles were loaded for all samples. D/Immunofluorescence of myoferlin in sections of WT (left panel) and TgMyof (middle panel) muscles. A control in which the primary antibody was omitted is shown (right panel). Scale bar=50 µm. E/Weight of WT and TgMyof muscles at 9 months of age. The values of TgMyof muscles are not statistically significant compared with WT mice (p>0.05). F/Histological analysis of 9-month-old male TgMyof showed no abnormality. Scale Bar=50 µm.