Figure 1.

Dystrophin protein abundance and localization. Dystrophin was assayed in the diaphragm, psoas, and longissimus in muscles taken from healthy pigs and pigs containing an arginine to tryptophan (R1958W) polymorphism in exon 41 of the dystrophin gene, DMD. A) By Western blot, dystrophin protein abundance was decreased by 70% in muscles from diseased animals compared with muscles from healthy animals. B) Representative ×400 images resulting from IHC targeted to dystrophin. C) Following fluorescence quantification, dystrophin abundance by IHC was significantly decreased in the diaphragm and longissimus (P<0.05), but failed to reach significance in the psoas, in part because variability in the control animals was high and in part because the magnitude of change in the psoas was not as substantial as in the diaphragm and longissimus. D) Dystrophin transcript abundance at 5 locations along DMD in the diaphragm. SNP, single-nucleotide polymorphism. E) Dystrophin transcript abundance at 5 locations along DMD in the psoas. F) Dystrophin transcript abundance at 5 locations along DMD in the longissimus. *P ≤ 0.05 vs. corresponding control.