Table 1.
Parameters recorded from myoblasts derived from patient 1 and 2.
| Patient 1 | Patient 2 | ||
|---|---|---|---|
| Cell capacitance (pF) | 53.11 ± 6.1 (n = 100) | 52.23 ± 5.7 (n = 63) | |
| Current Density (pA/pF) | −35.45 ± 5.5 (n = 100) | −34.15 ± 6.05 (n = 63) | |
| τslow (ms) | 7.44 ± 0.9 (n = 85) | 6.60 ± 0.7 (n = 50) | |
| τfast (ms) | 0.93 ± 0.06 (n = 100)* (p = 0.018) | 0.76 ± 0.04 (n = 63) | |
| Steady-state activation | V1/2 (mV) | −32,87 ± 1 (n = 13) | −30,69 ± 1,1 (n = 10) |
| k | 6,5 ± 0,4 (n = 13) | 6,7 ± 0,8 (n = 10) | |
| Steady-state fast inactivation | V1/2 (mV) | −73,86 ± 1,3* (n = 8, p = 0.0019) | −78,82 ± 1 (n = 9) |
| k | 7,7 ± 0,2 (n = 8) | 7,17 ± 0,3 (n = 9) | |
The number of cells considered for the τslow is smaller compared to the one of the other parameters as we eliminated the negative values that came out from the fitting analysis (in both the patients this elimination regarded about 15% of cells). The number of independent experiments were 17 for patient 1 and 15 for patient 2. *p < 0,05