Skip to main content
Figure 4 | Skeletal Muscle

Figure 4

From: Novel mutations in NEB cause abnormal nebulin expression and markedly impaired muscle force generation in severe nemaline myopathy

Figure 4

Weakness in severe NM with greatly reduced nebulin levels is associated with reduced force generation with increased tension cost and slower force redevelopment. Skinned myofibers from control patients (Ctrl) and patient 16-4 (16-4) reveal (A) a large reduction in maximal tension measured at -log[Ca2+] (pCa) 4.5, (B) an increase in tension cost and (C) a large reduction in the rate constant of force development (ktr). Control myofibers were taken from three normal quadriceps biopsies, and analysis was limited to type 2 fibers, since the patient 16-4 biopsy expressed mainly type 2 myosin heavy chains. Values are the means ± SEM of measurements from five myofibers. *P < 0.05.

Back to article page