Fig. 7
From: Recessive mutations in muscle-specific isoforms of FXR1 cause congenital multi-minicore myopathy
Ultrastructural evaluation of delACAG granules. a Representative TEM images of granules of different size exclusively observed in gastrocnemius fibers from delACAG mice. Granules are often located in close proximity to mitochondria. n = 3(wt), 4(delACAG), 2(dupA) mice. Scale bars 2 µm (left panel), 200 nm (rest of panels). b Representative TEM images of ring-shaped granules from V-4 differentiated myotubes. These structures were not present in control differentiated myotubes. Multiple myotubes were analyzed. Scale bars 2 µm (left panel), 200 nm (rest of panels). c Anti-FXR1P immunogold labeling of ultrathin sections from gastrocnemius of delACAG mice showing localization of the mutant protein in the outer electrodense layer of delACAG granules. n = 3. Scale bar 200 nm. d No-primary antibody control demonstrating no immunogold labeling in granules from delACAG mice. Scale bar 200 nm
