Table 3. Outcome measures for 30–33 week old omigapil and vehicle treated dy2J mice after 17.5 weeks show significant improvements in respiratory rate and fibrosis in dy2J 0.1 mg/kg omigapil treated mice.
| Measurement | dy2J vehicle | dy2J Omigapil 0.1 mg | dy2J Omigapil 1 mg | Significantly different mean/medians | |||
| N | Mean ± SD | N | Mean ± SD | N | Mean ± SD | ||
| %FS | 7 | 34±1 | 7 | 33±3 | 7 | 34±1 | NONE |
| %EF | 7 | 64±2 | 7 | 63±2 | 7 | 64±2 | NONE |
| Heart rate (BPM) | 7 | 548±18 | 7 | 527±34 | 7 | 563±63 | NONE |
| PA velocity (mm/s) | 7 | 724±88 | 7 | 694±74 | 7 | 687±93 | NONE |
| Ao velocity (mm/s) | 7 | 924±105 | 7 | 917±106 | 7 | 942±73 | NONE |
| E/A ratio | 7 | 1.66±0.05 | 7 | 1.68±0.11 | 5 | 1.81±0.15 | NONE |
| Horizontal activity* | 7 | 619±111; 562 (487–748) | 7 | 906±290; 778(682–1332) | 7 | 696±258; 802 (203–1285) | NONE |
| Total distance (cm)* | 7 | 63±20; 70 (34–84) | 7 | 163±110; 112(69–324) | 7 | 128±97; 115 (15–296) | NONE |
| Movement time(second)* | 7 | 9±3; 10 (5–13) | 7 | 23±14; 17 (11–45) | 7 | 19±14; 18 (2–43) | Vehicle vs. 0.1 mg (p = 0.026) |
| Rest time(second)* | 7 | 591±3; 590 (587–595) | 7 | 577±14; 583(555–589) | 7 | 581±14; 582 (557–598) | Vehicle vs. 0.1 mg (p = 0.026) |
| Vertical activity* | 7 | 0±0; 0 (0–0) | 7 | 0.1±0.4; 0 (0–1) | 7 | 0±0; 0 (0–0) | NONE |
| GSM forelimb (KGF) | 7 | 0.090±0.010 | 7 | 0.084±0.012 | 7 | 0.086±0.010 | NONE |
| Normalized GSM forelimb(KGF/kg) | 7 | 4.239±0.483 | 7 | 4.318±0.603 | 7 | 4.321±0.621 | NONE |
| Body weight (g) | 7 | 21.4±2.6 | 7 | 19.5±2.3 | 7 | 20.3±3.2 | NONE |
| Respiratory rate (bpm) | 7 | 371±18 | 7 | 396±11 | 7 | 402±19 | Vehicle vs. 0.1 mg (p = 0.026)Vehicle vs. 1 mg (p = 0.006) |
| Heart weight/BW | 6 | 4.34±0.36 | 7 | 4.38±0.40 | 7 | 4.51±0.53 | NONE |
| Spleen weight/BW | 7 | 3.34±0.55 | 7 | 3.13±0.63 | 7 | 3.22±0.62 | NONE |
| Gastroc Weight/BW | 7 | 2.25±0.47 | 7 | 2.19±0.58 | 7 | 2.17±0.48 | NONE |
| Soleus weight/BW | 7 | 0.24±0.10 | 7 | 0.28±0.05 | 7 | 0.30±0.07 | NONE |
| TA weight/BW | 6 | 1.08±0.32 | 7 | 1.28±0.18 | 7 | 1.28±0.27 | NONE |
| Hindlimb maximal force | 7 | 223±31 | 7 | 220±41 | 7 | 223±46 | NONE |
| Hindlimb specific force | 7 | 184±28 | 7 | 193±27 | 7 | 177±22 | NONE |
| % fibrosis – gastroc | 7 | 20.6±2.6 | 7 | 16.4±2.0 | 7 | 17.2±3.3 | Vehicle vs. 0.1 mg (p = 0.030) |
| % fibrosis - diaphragm | 7 | 14.7±0.7 | 7 | 9.4±1.7 | 7 | 12.2±2.1 | Vehicle vs. 0.1 mg (p<0.001)Vehicle vs. 1 mg (p = 0.032)0.1 mg vs. 1 mg (p = 0.013) |
| % area with degeneratingfibers – gastroc. | 7 | 10.1±3.1 | 7 | 3.0±0.7 | 7 | 5.8±3.8 | Vehicle vs. 0.1 mg (p = 0.001)Vehicle vs. 1 mg (p = 0.037) |
| % Centralized nucleifiber-gastroc* | 7 | 36.4±3.2; 38.0 (30.5–39.5) | 7 | 28.2±3.0; 27.3 (25.3–33.4) | 7 | 29.0±4.9; 27.6 (22.7–36.1) | Vehicle vs. 0.1 mg (p = 0.0120)Vehicle vs. 1 mg (p = 0.0376) |
| % Centralized nucleifiber-diaphragm* | 7 | 13.6±1.5; 13.3 (12.0–16.6) | 6 | 13.2±1.9; 12.9 (11.1–16.5) | 7 | 12.8±1.5; 13.1 (10.9–14.9) | NONE |
| % apoptosis nuclei per field* | 3 | 36.3±4.1; 38.0 (31.5–39.2) | 4 | 5.9±1.1; 5.6 (4.7–7.5) | 4 | 4.5±1.7; 4.3 (2.9–6.9) | NONE |
*
Non-parametric comparison of medians; data expressed as mean ± SD; median (range).
Abbreviations: %FS – percent fractional shortening, %EF- percent ejection fraction, BPM- beats per minute, bpm – breaths per minute, SD – standard deviation, PA – pulmonary artery, AO – aortic, E/A – ratio of mitral valve E and A wave velocities, GSM – grip strength meter, BW- body weight, Gastroc – gastrocnemius, TA – tibialis anterior, KGF.