. 2017 Mar 8;5:31–42. doi: 10.1016/j.omtm.2017.02.002

Table 1.

Assessment of Respiratory Function

Post-injection Time Point^a	T18		T26		T39		T52
Post-injection Time Point^a	AAV Treated	FKRP^P448L Mutant	AAV Treated	FKRP^P448L Mutant	AAV Treated	FKRP^P448L Mutant	AAV Treated	FKRP^P448L Mutant
F (Breaths/min)

1 week	248.56 ± 5.86	253.70 ± 3.36	258.55 ± 5.44	260.36 ± 6.12	279.01 ± 13.32	271.63 ± 5.22	276.15 ± 5.93	270.64 ± 8.90
13 weeks	273.73 ± 6.08	275.58 ± 8.15	244.77 ± 1.15	268.89 ± 9.32	284.20 ± 18.54	289.85 ± 10.91	262.74 ± 12.80	276.03 ± 22.34
Percent change	10.1	8.6	−5.3	3.3	1.9	6.7	−4.9	2.0

TV (mL)

1 week	0.16 ± 0.01	0.16 ± 0.01	0.17 ± 0.01	0.18 ± 0.00	0.25 ± 0.02	0.24 ± 0.02	0.22 ± 0.02	0.21 ± 0.02
13 weeks	0.26 ± 0.02	0.23 ± 0.01	0.27 ± 0.03	0.25 ± 0.01	0.26 ± 0.03	0.24 ± 0.01	0.21 ± 0.01	0.21 ± 0.02
Percent change	60.1	45.6	59.1	34.1	1.7	−0.2	−5.4	2.0

MV (mL)

1 week	40.29 ± 2.30	39.78 ± 1.67	43.13 ± 1.80	47.98 ± 1.66	71.15 ± 8.53	64.26 ± 6.34	61.92 ± 5.74	57.05 ± 6.33
13 weeks	71.26 ± 6.96	62.73 ± 5.06	64.76 ± 6.55	67.01 ± 3.29	74.10 ± 10.75	68.23 ± 5.39	55.52 ± 4.86	60.45 ± 10.00
Percent change	76.9	57.7	50.1	39.7	4.1	6.2	−10.3	5.9

PIF (mL/s)

1 week	5.98 ± 0.19	5.95 ± 0.23	6.34 ± 0.18	6.78 ± 0.35	6.87 ± 0.34	6.75 ± 0.45	5.78 ± 0.45	5.68 ± 0.51
13 weeks	6.73 ± 0.53	6.32 ± 0.25	7.08 ± 0.61	6.38 ± 0.37	6.40 ± 0.43	5.57 ± 0.18	5.10 ± 0.15	5.00 ± 0.35
Percent change	12.7	6.4	11.7	−6.0	−6.8	−17.5	−11.8	−12.0

PEF (mL/s)

1 week	3.38 ± 0.18	3.92 ± 0.14	3.94 ± 0.23	4.13 ± 0.25	4.01 ± 0.32	4.08 ± 0.31	3.57 ± 0.23	3.42 ± 0.18
13 weeks	4.12 ± 0.32	4.07 ± 0.19	4.05 ± 0.30	3.81 ± 0.20	4.08 ± 0.37	4.00 ± 0.13	3.35 ± 0.17	3.61 ± 0.21
Percent change	21.9	3.9	2.6	−7.9	1.6	−2.1	−6.2	5.5

Respiratory function parameters in AAV-treated and FKRP^P448L mutant mice for T18, T26, T39, and T52 cohorts at post-injection time point intervals of 1 and 13 weeks. F, breathing frequency; MV, minute volume; PEF, peak expiratory flow; PIF, peak inspiratory flow; TV, tidal volume.

Percent change from 1 to 13 weeks.