Skip to main content

Table 1 Phenotypic measures in NBD-treated and untreated GRMD and normal dogs

From: NBD delivery improves the disease phenotype of the golden retriever model of Duchenne muscular dystrophy

Test

Normal (Untreated) (n = 8)

Normal (NBD-treated) (n = 3)

GRMD (Untreated) (n = 10)

GRMD (NBD-treated) (n = 6)

Age (months)

Age (months)

Age (months)

Age (months)

 

3

6

2

6

3

6

2

6

Body Mass (kg)

8.46 ± 0.632*

17.80 ± 0.441***,2***

4.99 ± 1.83

21.10 ± 3.151*

7.36 ± 1.29

13.40 ± 2.201***

3.60 ± 0.27

12.70 ± 2.811**

Absolute Tetanic Flexion Force (N)

9.51 ± 1.072***

23.19 ± 2.201***,2***

4.52 ± 1.82

25.75 ± 2.181**

3.85 ± 1.13

7.85 ± 1.981***

2.05 ± 0.58

6.79 ± 1.131***

Body-mass Corrected Tetanic Flexion Force (N/kg)

1.12 ± 0.102***

1.30 ± 0.131*,2***

0.90 ± 0.042**

1.24 ± 0.19

0.53 ± 0.14

0.62 ± 0.18

0.57 ± 0.15

0.55 ± 0.13

Absolute Tetanic Flexion Torque (Nm)

1.08 ± 0.142***

3.22 ± 0.351***,2***

0.42 ± 0.24

3.65 ± 0.431**

0.43 ± 0.14

1.03 ± 0.231***

0.17 ± 0.06

0.89 ± 0.181***

Body-mass Corrected Tetanic Flexion Torque (Nm/kg)

0.13 ± 0.012***

0.18 ± 0.021***,2***

0.08 ± 0.02

0.17 ± 0.02

0.06 ± 0.02

0.08 ± 0.02

0.05 ± 0.01

0.07 ± 0.021*

Absolute Tetanic Extension Force (N)

22.56 ± 2.762***

46.43 ± 8.441***,2***

13.46 ± 3.26

42.17 ± 7.461*

12.73 ± 2.12

18.47 ± 10.11

7.50 ± 2.09

34.30 ± 9.651**,2*

Body-mass Corrected Tetanic Extension Force (N/kg)

2.68 ± 0.382**

2.61 ± 0.492**

2.88 ± 1.10

2.00 ± 0.22

1.80 ± 0.52

1.56 ± 0.92

2.11 ± 0.70

2.70 ± 0.652*

Absolute Tetanic Extension Torque (Nm)

2.55 ± 0.372***

6.44 ± 1.241***,2***

1.20 ± 0.41

6.02 ± 1.381*

1.42 ± 0.23

2.46 ± 1.28

0.62 ± 0.16

4.55 ± 1.491**,2*

Body-mass Corrected Tetanic Extension Torque (Nm/kg)

0.30 ± 0.042***

0.36 ± 0.072***

0.25 ± 0.08

0.28 ± 0.04

0.20 ± 0.05

0.18 ± 0.10

0.21 ± 0.07

0.36 ± 0.081*,2**

ECD (%; 1-10)

13.68 ± 3.97

8.29 ± 2.881*,2**

13.36 ± 2.65

14.47 ± 7.31

13.97 ± 10.52

28.96 ± 18.07

16.12 ± 5.88

32.04 ± 13.46

ECD (%; 1-30)

26.80 ± 5.922*

17.86 ± 5.201*,2***

33.44 ± 10.3

23.25 ± 17.4

36.51 ± 11.49

54.30 ± 14.731*

38.67 ± 10.34

54.48 ± 13.64

TTJ Angle (o)

167.9 ± 3.942***

160.9 ± 3.401**,2*

161.7 ± 7.64

158.3 ± 2.08

159.40 ± 3.72

151.30 ± 10.19

157.00 ± 9.80

160.17 ± 8.84

Hip (Resting)

99.50 ± 5.10

104.4 ± 5.76

99.33 ± 1.15

103.0 ± 11.4

103.4 ± 8.96^

109.6 ± 8.02

103.7 ± 2.94

100.2 ± 6.012*

Maximum Hip Flexion (o)

55.50 ± 8.86

59.00 ± 12.60

48.67 ± 7.77

53.33 ± 10.12

54.40 ± 7.50^

68.60 ± 16.15

44.67 ± 5.47

46.33 ± 3.502**

Pelvic Angle (o)

43.75 ± 5.12

36.14 ± 5.982**

38.33 ± 1.53

40.33 ± 5.03

40.00#

48.30 ± 5.54

41.00 ± 6.81

36.83 ± 5.122**

CS Circumference (mm/kg)

NA

2.09 ± 0.402***

NA

2.30 ± 0.49

NA

4.03 ± 1.04

NA

3.67 ± 0.65

  1. Functional studies, chiefly TTJ tetanic force and pelvic limb joint angles, were assessed to demonstrate therapeutic benefit of NBD in GRMD dogs and potential effects in wild type dogs. N = Newtons; Nm = Newton meters; ECD = Eccentric contraction deficit; CS = Cranial Sartorius; TTJ = tibiotarsal joint.
  2. ^Data for only five GRMD dogs available at 3 mos.
  3. #Data for only one GRMD dog available at 3 mos.
  4. 1Significantly different from 2/3 to 6 mos, same genotype; *p < 0.05, **p < 0.01; ***p < 0.001.
  5. 2Significantly different from GRMD natural history; *p < 0.05, **p < 0.01; ***p < 0.001.