Table 1.
Echocardiographic phenotype in single transgenic and bigenic MYOZ2 wild-type and mutant mice
| NTG | MYOZ2WT | MYOZ2S48P | MYOZ2I246M | MYOZ2−/− | MYOZ2+/− | MYOZ2+/− :MYOZWT | MYOZ2+/− :MYOZ2I246M | P-value (ANOVA) | |
|---|---|---|---|---|---|---|---|---|---|
| n | 58 | 17 | 8 | 16 | 10 | 11 | 13 | 12 | NA |
| Sex (M/F) | 38/20 | 6/11 | 3/5 | 8/8 | 5/5 | 6/5 | 6/7 | 5/7 | 0.227 |
| Age (months) | 9.8 ± 3.4 | 11.6 ± 7.6 | 16.6 ± 5.9 | 12.3 ± 6.6 | 9.5 ± 2.0 | 9.5 ± 2.8 | 9.5 ± 5.5 | 9.5 ± 3.4 | 0.243* |
| Age (months) | 9.8 ± 3.4 | 11.6 ± 7.6 | 16.6 ± 5.9 | 12.3 ± 6.6 | 9.5 ± 2.0 | 9.5 ± 2.8 | 9.5 ± 5.5 | 9.5 ± 3.4 | 0.243* |
| Body weight (g) | 33.1 ± 4.1 | 34.6 ± 4.2 | 36.6 ± 3.8 | 36.6 ± 5.1 | 36.6 ± 5.3 | 35.3 ± 8.6 | 34.5 ± 4.2 | 35.6 ± 6.5 | 0.003* |
| Heart rate (b.p.m.) | 561.7 ± 86.2 | 650.0 ± 61.6* | 623.8 ± 76.5 | 601.3 ± 62.9 | 528.0 ± 109.3 | 5773 ± 103.6 | 580 ± 77.9 | 586.7 ± 92.8 | 0.010 |
| IVST (mm) | 0.97 ± 0.11 | 1.04 ± 0.14 | 1.18 ± 0.10**,*** | 1.10 ± 0.12** | 0.85 ± 0.10 | 0.90 ± 0.09 | 0.88 ± 0.07 | 1.03 ± 0.05****,***** | <0.0001 |
| PWT (mm) | 0.93 ± 0.12 | 0.93 ± 0.11 | 1.10 ± 0.09**,***,****** | 0.99 ± 0.12 | 0.84 ± 0.08 | 0.89 ± 0.10 | 0.83 ± 0.10 | 0.97 ± 0.10****,***** | <0.0001 |
| LVEDD (mm) | 2.85 ± 0.35 | 3.04 ± 0.31 | 3.12 ± 0.22 | 3.17 ± 0.22** | 3.47 ± 0.22** | 3.40 ± 0.26** | 3.40 ± 0.30** | 3.29 ± 0.33** | <0.0001 |
| LVESD (mm) | 1.02 ± 0.20 | 1.02 ± 0.20 | 1.06 ± 0.17 | 1.01 ± 0.15 | 1.34 ± 0.28** | 1.25 ± 0.19** | 1.13 ± 0.19 | 1.12 ± 0.30 | 0.0004 |
| LV mass (mg) | 87.9 ± 17.0 | 101.9 ± 15.4 | 132.1 ± 15.0**,***,****** | 116.9 ± 7.6**,*** | 100.4 ± 19.8 | 105.5 ± 16.7** | 99.1 ± 13.3 | 117.5 ± 17.2**,***** | <0.0001 |
| FS (%) | 64.3 ± 5.5 | 66.5 ± 5.2 | 66.0 ± 5.01 | 68.0 ± 3.4 | 61.4 ± 7.1 | 63.2 ± 4.9 | 66.7 ± 5.1 | 66.2 ± 7.0 | 0.065 |
| Vcf (mm/s) | 34.6 ± 11.4 | 44.6 ± 12.6 | 41.1 ± 8.1 | 46.1 ± 9.4** | 37.8 ± 9.6 | 41.6 ± 6.7 | 46.6 ± 9.2** | 42.7 ± 10.3 | 0.002 |
| Mitral inflow E/A | 1.17 ± 0.18 | 1.13 ± 0.16 | 1.13 ± 0.21 | 1.00 ± 0.23 | 2.21 ± 0.46** | 1.54 ± 0.26** | 1.44 ± 0.33 | 2.00 ± 0.08**,***** | <0.0001 |
| Aortic outflow velocity (m/s) | 1.11 ± 0.44 | 1.37 ± 0.15 | 1.32 ± 0.23 | 1.11 ± 0.20 | 1.22 ± 0.24 | 1.25 ± 0.22 | 1.18 ± 0.19 | 1.20 ± 0.20 | 0.022* |
n, number; NTG, non-transgenic; MYOZ2, myozenin 2; M/F, male/female; g, gram; b.p.m., beats per minutes; IVST, interventricular septal thickness; PWT, posterior wall thickness; LVEDD, left ventricular end diastolic diameter; LVESD, left ventricular end systolic diameter; LV mass, left ventricular mass; FS, fractional shortening.
*P value by the Kruskal–Wallis rank test because of departure from a normal distribution.
**P < 0.05 by the Bonferroni pairwise multiple comparison test when compared with non-transgenic.
***P < 0.05 vs. Myoz2WT.
****P < 0.05 by the Bonferroni pairwise multiple comparison test between Myoz2+/−:Myoz2I246M and Myoz2+/−:Myoz2WT.
*****P < 0.05 vs. Myoz2+/−:MYOZ2WT.
******P < 0.05 by the Bonferroni pairwise multiple comparison test between Myoz2S48P or Myoz2I246M and Myoz2WT.