Fig. 5. Nemaline rod bodies, sarcomeric ultrastructure, and thin filament length.

a Electron micrographs of intact EDL muscles (left) and soleus muscle (right) in WT (top), Compound-Het (middle) and Neb^S6336I How (bottom). Nemaline rod bodies (yellow arrows) are found in both EDL and soleus of Compound-Het and in only EDL of Neb^S6366I HOM mice. Inset in left middle panel shows enlarged rod bodies. In soleus muscle sarcomere disorganization was frequently seen in both Compound-HET and Neb^S6366I HOM mice. Yellow arrows: rod bodies; blue arrows: altered/damaged Z-disks. Rod body size (b) and fractional area of rod bodies (c) in EDL and soleus. b *p = 0.03. c **p = 0.0059. Values are means ± SEM. Four mice per genotype were used with ~5 fibers per muscle examined. The mean value for each mouse is plotted. d Thin filament lengths are slightly but significantly longer in Compound Het and Neb^S6366I Hom EDL muscle. Measurements made in SL range: 2.5–3.0 µm. (See Supplementary Fig. 4 for images and additional data, including confirmations by super-resolution optical microscopy.) b–d Ordinary one-way ANOVA without matching or paring. A posthoc multiple testing comparison with multiple testing correction (Tukey) was performed. d ****p < 0.0001. n(WT) = 81 measurements from 18 fibers from 6 mice; n(Hom) = 71 measurements from 18 fibers from 6 mice; n(Compound HET) = 103 measurements from 18 fibers from 6 mice. Horizontal lines are mean ± SEM. Source data are provided as a Source Data file.