Table 1.
Mut R719W norm. | Mut R723G norm. |
Mut I736T norm. |
Controls norm. | Controls abs. values |
Units for abs. values | |||||
---|---|---|---|---|---|---|---|---|---|---|
H27 | H28 | H19 | H20 | 20°C | 10°C | |||||
Fmax | 1.43∗ ± 0.02 (14) | 1.19∗ ± 0.01 (7) | 1.22∗ ± 0.01 (14) | 0.97 ± 0.02 (7) | 1.01 ± 0.01 (20) | 1.00 ± 0.01 (45) | 140.86 ± 1.25 (45) | 108.94 ± 5.31 (36) | kN/m2 or kPa | |
kredev | 1.00 ± 0.05 (14) | 1.11 ± 0.04 (7) | 1.09 ± 0.06 (14) | 0.91 ± 0.04 (7) | 0.97 ± 0.03 (20) | 1.00 ± 0.04 (45) | 2.69 ± 0.06 (45) | 0.27 ± 0.02 (36) | s−1 | |
Fiber-ATPase | 1.21∗ ± 0.02 (14) | 0.97 ± 0.04 (6) | 0.98 ± 0.02 (12) | 0.97 ± 0.05 (6) | 1.03 ± 0.03 (11) | 1.00 ± 0.03 (21) | 0.95§ ± 0.02 (21) | n.d. | ||
vmax | 1.10 ± 0.11 (6) | 1.07 ± 0.04 (8) | 1.17∗ ± 0.04 (9) | 0.95 ± 0.04 (11) | 1.00 ± 0.03 (10) | 1.00 ± 0.03 (7) | 3246.00 ± 86.79 (7) | 329.90 ± 8.61 (10) | (nm/HS)s−1 | |
Fiber stiffness in rigor at 5°C† | see Table 2 | 1.43∗ ± 0.21 (5) | 1.59∗ ± 0.15 (6) | 1.09 ± 0.09 (7) | 1.15 ± 0.14 (10) | 1.00 ± 0.15 (6) | n.d. | 17.7 ± 2.7 (6) | or kPa × HS/nm | |
Active fiber stiffness† | see Table 2 | 1.32∗ ± 0.10 (10) | 1.43∗ ± 0.19 (6) | 0.90 ± 0.04 (7) | 0.97 ± 0.08 (11) | 1.00 ± 0.13 (9) | n.d. | 13.2 ± 1.7 (9) | or kPa × HS/nm | |
abs. values | abs. values | |||||||||
y0 (nm/HS)‡ at 10°C | see Table 2 | 7.70 ± 0.74 (9) | n.d. | n.d. | 7.76 ± 0.44 (7) | n.d. | 8.53 ± 0.42 (6) |
abs., absolute; HS, half-sarcomere; n.d., not determined; norm., normalized.
Isometric force at 10°C of fibers with mutation R723G (H27) and I736T (H20) was 1.23 ± 0.05 (n = 9) times and 0.91 ± 0.08 (n = 11) times the force of control fibers, respectively. Values in parentheses indicate number of fibers studied, data are mean values ± SE. All values are normalized to control fibers that were measured in the same time period. All normalized values of Fmax, kredev, fiber-ATPase, and vmax shown here were measured at 20°C, rigor stiffness was measured at 5°C, active stiffness and y0 were determined at 10°C.
Significant changes compared to controls.
Fiber stiffness in rigor and isometric contraction measured at fastest speed of length change (∼3–5 × 103 (nm/half-sarcomere)s−1 for rigor and 2–5 × 103 (nm/half-sarcomere)s−1 for active stiffness).
y0 and active stiffness of the control fibers were measured on a different set of fibers with Fmax = 112.95 ± 0.11 kPa.
ATPase activity was calculated based on ADP generated, fiber volume and an S1-content of 154 pmol S1/μL fiber volume (50).