TABLE I.
Comparison of preclinical outcome measures.
| Control C57 Mean ±SEM (n) |
Untreated Het Mean ±SEM (n) |
EL treated Mean ±SEM (n) |
LS treated Mean ±SEM (n) |
P-value | Best het group or closest to C57 | |||
|---|---|---|---|---|---|---|---|---|
| ANOVA | Dunnett Untreated vs EL | Dunnett Untreated vs LS | ||||||
| Body Weight (g) | 28.3 ± 0.5 (18) | 32.2 ± 0.8 (18) | 30.1 ± 0.5 (17) | 31.0 ± 1.1 (18) | 0.00446 | 0.1438 | 0.5684 | EL treated |
| Grip Strength (N) | 1.5 ± 0.05 (18) | 1.1 ± 0.07 (18) | 1.1 ± 0.07 (17) | 1.2 ± 0.07 (18) | < .0001 | 0.798 | 0.8083 | LS treated |
| GS/BW (N/mg) * | 53.5 ± 1.6 (18) | 35.3 ± 2.5 (18) | 34.9 ± 1.9 (17) | 38.3 ± 1.9 (18) | < .0001 | 0.997 | 0.5852 | LS treated |
| Heart Weight (g) | 0.14 ± 0.004 (18) | 0.17 ± 0.006 (18) | 0.13 ± 0.003 (17) | 0.15 ± 0.005 (17) | < .0001 | < .0001 | 0.0079 | EL treated |
| HW/BW (mg/g) * | 5.0 ± 0.1 (18) | 5.2 ± 0.1 (18) | 4.5 ± 0.1 (17) | 4.7 ± 0.1 (17) | < .0001 | < .0001 | 0.0058 | EL treated |
| Heart Rate (bpm) | 592 ± 16 (18) | 605 ± 20 (18) | 613 ± 18 (17) | 591 ± 23 (18) | 0.83173 | 0.9797 | 0.9256 | LS treated |
| QT (ms) * | 51.1 ± 2.2 (18) | 51.2 ± 1.8 (18) | 47.8 ± 2.7 (17) | 49.9 ± 4.0 (18) | 0.82311 | 0.7341 | 0.9723 | EL treated |
| QTc (ms) | 49.2 ± 1.8 (18) | 52.0 ± 2.9 (18) | 49.7 ± 0.8 (17) | 52.5 ± 3.4 (18) | 0.71999 | 0.849 | 0.9985 | EL treated |
| MID_PeakSysSR_Mean (endocardial circumferential S−1) | ND | 0.42 ± 0.04 (12) | 0.50 ± 0.05 (12) | 0.52 ± 0.05 (11) | 0.25608 $ | 0.3292 | 0.2187 | LS treated |
| BASE-PeakSysSR_Mean (endocardial circumferential S−1) * | ND | 0.37 ± 0.03 (12) | 0.40 ± 0.03 (11) | 0.49 ± 0.05 (11) | 0.08108 $ | 0.8309 | 0.0584 | LS treated |
| Cardiac Fdev (mN/mm−2) * | 11.6 ± 1.9 (17) | 9.5 ± 2.2 (16) | 4.9 ± 0.7 (12) | 15.4 ± 4.4 (14) | 0.07549 | 0.4972 | 0.2736 | LS treated |
| Cardiac Fmax (mN/mm−2) | 16.6 ± 2.7 (17) | 16.2 ± 3.8 (16) | 10.0 ± 3.3 (12) | 18.2 ± 4.8 (14) | 0.49797 | 0.5318 | 0.965 | LS treated |
| Diaphragm spec. force * (mN/mm−2) | 142.2 ± 10.0 (16) | 114.8 ± 7.9 (18) | 116.4 ± 8.2 (16) | 103.1 ± 8.3 (18) | 0.01863 | 0.9985 | 0.6352 | EL treated |
| Diaphragm Absolute Recovery (mN/mm−2) * | 13.5 ± 2.2 (15) | 7.9 ± 0.9 (17) | 10.1 ± 1.0 (15) | 8.5 ± 1.2 (16) | 0.02681 | 0.5241 | 0.9773 | EL treated |
| Diaphragm Relative Recovery (%) | 31.7 ± 4.4 (15) | 17.8 ± 1.5 (17) | 21.8 ± 1.2 (15) | 21.0 ± 2.0 (16) | 0.00213 | 0.5442 | 0.6897 | EL treated |
| EDL tet (mN/mm−2) * | 443 ± 21 (18) | 352 ± 19 (13) | 420 ± 28 (16) | 384 ± 34 (15) | 0.09242 | 0.203 | 0.7521 | EL treated |
| ecc1 (% tet) | 63 ± 2 (17) | 55 ± 6 (12) | 62 ± 4 (14) | 59 ± 5 (14) | 0.52856 | 0.4953 | 0.8805 | EL treated |
| ecc2 (% tet) * | 30 ± 3 (17) | 21 ± 5 (12) | 28 ± 4 (14) | 30 ± 5 (13) | 0.41442 | 0.5474 | 0.3446 | LS treated |
| ecc5 (% tet) | 10 ± 1 (17) | 5 ± 1 (12) | 8 ± 2 (13) | 12 ± 3 (13) | 0.13579 | 0.648 | 0.068 | LS treated |
| ecc10 (% tet) | 4 ± 1 (17) | 2 ± 1 (12) | 3 ± 1 (13) | 6 ± 2 (12) | 0.15307 | 0.6245 | 0.0729 | LS treated |
| Post-rest ecc 11 (% tet) | 8 ± 1 (15) | 3 ± 1 (10) | 7 ± 2 (13) | 8 ± 2 (11) | 0.1744 | 0.3851 | 0.1653 | LS treated |
| ((ecc11 - ecc10) / ecc10) x 100% (% ecc10) * | 128 ± 17 (15) | 107 ± 14 (10) | 130 ± 16 (13) | 153 ± 37 (11) | 0.59542 | 0.823 | 0.3847 | LS treated |
| Quad IgG (% CSA) * | 0.6 ± 0.1 (18) | 9.7 ± 1.0 (18) | 8.9 ± 0.9 (17) | 9.9 ± 1.5 (18) | < .0001 | 0.8912 | 0.9991 | EL treated |
| Heart IgG (% CSA) * | 0.1 ± 0.03 (18) | 2.7 ± 0.4 (18) | 1.7 ± 0.5 (16) | 1.3 ± 0.3 (18) | < .0001 | 0.0701 | 0.0059 | LS treated |
| Dia IgG (% CSA) * | 0.5 ± 0.1 (18) | 14.5 ± 0.6 (18) | 11.8 ± 0.8 (17) | 10.8 ± 0.8 (18) | < .0001 | 0.0117 | 0.0003 | LS treated |
| Abd IgG (% CSA) * | 0.5 ± 0.1 (18) | 8.8 ± 0.7 (18) | 8.3 ± 1.6 (17) | 6.3 ± 0.6 (18) | < .0001 | 0.9717 | 0.1225 | LS treated |
*
indicates independent parameters used for the stochastic analyses
$
indicates ANOVA P-values calculated without a C57BL/10 wild-type control group
Measurements for 26 parameters from untreated het, LS treated and EL treated groups, compared with control C57 wild-type mice. ANOVA and Dunnett post-hoc P-values are shown for each parameter. The group with the closest value to wild-type or the best value if wild-type was not included (for MRI) is bolded and listed in the last column. P-values of < 0.05 are underlined.