Fig. 8
From: Generation of a novel mouse model of nemaline myopathy due to recurrent NEB exon 55 deletion
Nebulin distribution and localization. Double Immunofluorescence labelling for Neb N-terminus and α-actinin in n = 4 WT and HOM male Hmz-NebΔExon55 TA muscle. (A) Cross section indicates the proportion of higher nebulin expressing fibers is reduced in HOM mice. (B) Confocal imaging of longitudinal sections demonstrates that nebulin’s localization is normal in HOM mice. Note that nebulin expression in the Hmz muscle appears “thicker”, possibly as a result of the generally thickening of the Z band seen in these mice