Table 7.

Plethysmography—long-term tirasemtiv treatment

Week 0cWeek 4d# of miceAge of mice in months
Acta1(Wt)—vehicle 
Respiratory rate (per minute) 315 ± 50 330 ± 17 255 ± 53 310 ± 24   
Tidal volume (ml/kg) 9.5 ± 2.2 14.6 ± 4 8.6 ± 1.2 13 ± 2.3   
Minute volume (ml/kg/minute) 3,027 ± 844 4,863 ± 1511 2,164 ± 480 4,040 ± 565   
Acta1(Wt)—tirasemtiva 
Respiratory rate (per minute) 322 ± 44 331 ± 13 284 ± 50 310 ± 23   
Tidal volume (ml/kg) 8.9 ± 2 14.5 ± 3.2 7.9 ± 1.5 12.4 ± 1.7   
Minute volume (ml/kg/minute) 2,905 ± 698 4,782 ± 1,080 2,210 ± 450 3,848 ± 576   
Acta1(D286G)—vehicleb 13 
Respiratory rate (per minute) 357 ± 37 330 ± 23 319 ± 29 315 ± 25   
Tidal volume (ml/kg) 9.5 ± 2.1 14 ± 2.3 8.7 ± 1.7 14 ± 2.1   
Minute volume (ml/kg/minute) 3,396 ± 1,081 4,613 ± 812 2,724 ± 554 4,391 ± 768   
Acta1(D286G)—tirasemtiv 15 
Respiratory rate (per minute) 331 ± 47 324 ± 19 270 ± 47 277a ± 37   
Tidal volume (ml/kg) 8.9 ± 1.4 14.5 ± 3 9.2 ± 2 14.3 ± 2.4   
Minute volume (ml/kg/minute) 2,924 ± 641 4,711 ± 1,012 2,392 ± 448 3,933 ± 827   
Week 0cWeek 4d# of miceAge of mice in months
Acta1(Wt)—vehicle 
Respiratory rate (per minute) 315 ± 50 330 ± 17 255 ± 53 310 ± 24   
Tidal volume (ml/kg) 9.5 ± 2.2 14.6 ± 4 8.6 ± 1.2 13 ± 2.3   
Minute volume (ml/kg/minute) 3,027 ± 844 4,863 ± 1511 2,164 ± 480 4,040 ± 565   
Acta1(Wt)—tirasemtiva 
Respiratory rate (per minute) 322 ± 44 331 ± 13 284 ± 50 310 ± 23   
Tidal volume (ml/kg) 8.9 ± 2 14.5 ± 3.2 7.9 ± 1.5 12.4 ± 1.7   
Minute volume (ml/kg/minute) 2,905 ± 698 4,782 ± 1,080 2,210 ± 450 3,848 ± 576   
Acta1(D286G)—vehicleb 13 
Respiratory rate (per minute) 357 ± 37 330 ± 23 319 ± 29 315 ± 25   
Tidal volume (ml/kg) 9.5 ± 2.1 14 ± 2.3 8.7 ± 1.7 14 ± 2.1   
Minute volume (ml/kg/minute) 3,396 ± 1,081 4,613 ± 812 2,724 ± 554 4,391 ± 768   
Acta1(D286G)—tirasemtiv 15 
Respiratory rate (per minute) 331 ± 47 324 ± 19 270 ± 47 277a ± 37   
Tidal volume (ml/kg) 8.9 ± 1.4 14.5 ± 3 9.2 ± 2 14.3 ± 2.4   
Minute volume (ml/kg/minute) 2,924 ± 641 4,711 ± 1,012 2,392 ± 448 3,933 ± 827   
a

Tirasemtiv versus vehicle (P < 0.05).

b

Acta1(D286G) versus Acta1(wt) (P < 0.05).

c

Week 2 versus Week 0 (P < 0.05).

d

Week 4 versus Week 0 (P < 0.05).

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