Fig. 7
From: Generation of a novel mouse model of nemaline myopathy due to recurrent NEB exon 55 deletion
Hmz-NebΔExon55 nemaline rod formation and reduced thin filament length. (A) Modified gomori-trichrome stain on isopentane flash frozen Quad crossections from n = 2 ~ 2-month-old Het and HOM male Hmz-NebΔExon55 mice indicates that HOM mouse fiber size is reduced with apparent nemaline rod formation (yellow arrow). (B) TEM imaging of glutaraldehyde fixed longitudinal TA sections from n = 4 ~ 2-month-old WT and HOM Hmz-NebΔExon55 mice indicates clear nemaline rod formation throughout HOM tissue that is not present in WT. (C) Phalloidin staining ~ 3-month-old WT and HOM Hmz-NebΔExon55 thin filaments. At the bottom, a screen capture of a plot profile (yellow line) captured in ImageJ and how the plot profiles were fit with a rectangular gaussian in Fityk. (D) Thin filament length (TFL) measurements indicates that there is a shift to shorter TFL and sarcomeres in HOM animals. (E) WT/HOM t-test for TFL at sarcomere lengths of 2.4–2.8 μm indicates that there is a significant (P < 0.0001) TFL reduction from 1.06 μm in WT to 0.96 μm in HOM animals