Fig. 1.

Sarcomeric structure in EDL and soleus skinned skeletal muscle fibers of WT and nebulin KO mice. (A-D) EDL muscle; (E-H) soleus muscle. WT fibers have a regular structure with well-aligned sarcomeres. In KO fibers, sarcomeres are misaligned and the Z-disk structure is non-uniform, with nemaline bodies (arrows). (D,H) Measurement of longitudinal Z-disk displacement (ΔX, defined as longitudinal displacement of nearby Z-disk in adjacent myofibrils) in skeletal muscles. Note large Z-disk displacement in both EDL and soleus KO fibers. Scale bars: 0.5 μm (A,B,E,F); 2 μm (C,D,G,H). (Each result is from a different mouse. Fibers were stretched 80%.)