Table 2 Muscle contractility data of permeabilized fibers from nemaline myopathy patients with NEB mutations

Maximal active tension (mN/mm ² )

8.1 ± 1.5

18.8 ± 2.7

76.9 ± 10.0

90.9 ± 10.5

55.5 ± 7.1

102.2 ± 21.2

61.4 ± 12.2

Rate of tension redevelopment (k ₁ (s ^-1 ))

5.1 ± 0.4

4.5 ± 0.7

5.0 ± 0.5

3.4 ± 0.2

3.6 ± 0.2

4.0 ± 0.6

3.8 ± 0.5