Figure 5

Typical force traces of skinned myofibers from nemaline myopathy patients with nebulin mutations. Typical examples from force traces of a skinned muscle preparation from P1 from Group1 (in grey) and P9 from Group2 (in black). Myofibres are pre-activated by exposure to an activation solution at 1°C. By rapid switching to an activation solution at 20°C, the fibers are activated and force is generated. When the force trace reaches a plateau, the myofibres are slacked to 70% of their original length followed by a rapid restretch to the original length after 30 milliseconds (K_tr manouvre). When force generation has reached a plateau again, the muscle preparation is exposed to a calcium-free solution to induce relaxation.