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Fig. 2
Vcp deficiency results in a severe skeletal muscle myopathy. (a and b) Lateral view of control (a) and vcp morphants (b) showing birefringence at 48 hpf. scale bar: 450 μm. (c) Densitometric analysis of birefringence (n = 23/25). Individual samples are shown (two-sided Wilcoxon rank-sum test). (d and e) Skeletal muscle H&E-stained sagittal histological sections of MO1-control- (d) and MO1-vcp-injected embryos (e) at 72 hpf; scale bar: 100 μm. (f to g``) Transmission electron microscopy in control embryos (f to f``) and vcp morphants (g to g``) at 48 hpf. (f to f``) MO1-control-injected embryos show organized myofibrils and sarcomeric units with highly structured thick and thin filaments (f`) and mitochondria with many cristae (f``). (g to g``) VCP-deficient muscle cells present disorganized myofibrils with Z-disc alterations (arrow, g`), dysmorphic mitochondria (g``) and an accumulation of numerous vesicular bodies (double arrowheads, g); scale bar: 2.5 μm (f, g); scale bar: 1 μm (f` and f``, g` and g``).