Fig. 6
From: Generation of a novel mouse model of nemaline myopathy due to recurrent NEB exon 55 deletion
Hmz-NebΔExon55 muscle tissue weight and fiber size reduction. (A) Muscle tissue weights for ~ 3-month-old Hmz-NebΔExon55 WT, Het, and HOM mice. HOM animals have a reduction in tibialis anterior (TA), extensor digitorum longus (EDL), plantaris (Plant), gastrocnemius (Gast), and quadriceps (Quad) muscle compared to WT and HET littermates indicative of hypotrophy. Conversely, HOM animals have either no change or else a slight increase in soleus (SOL) and diaphragm (Dia) muscle weights compared to WT and HET littermates. (B) Measuring skeletal muscle fiber size in (n = 4) ~ 2-month-old Hmz-NebΔExon55 male mice TA muscle. Isopentane flash frozen horizontal TA sections were stained for dystrophin, a membrane associated protein. n = 8 representative 20X magnification immunofluorescence images were taken per sample and quantified in ImageJ for minimum Feret’s diameter. Imaging indicates a significant shift to smaller fiber size composition within HOM animals compared to WT