|
SYNE-1 E7854X
|
LMNA L380S
|
LMNA del.K32
|
LMNA R249W
|
---|
|
Myoblast
|
Myotube
|
Myoblast
|
Myotube
|
Myoblast
|
Myotube
|
Myoblast
|
Myotube
|
---|
N1-Giant
|
13.9 ± 2.2 (6)
|
33.5 ± 5.0 (5)
|
102.1 ± 8.8 (5)
|
429.5 ± 42.4 (5)
|
48.3 ± 9.4 (4)
|
161.7 ± 33.6 (5)
|
53.4 ± 10.0 (4)
|
90.7 ± 19.0 (5)
|
|
P < 0.001
|
P < 0.001
|
P < 0.001
|
P < 0.01
|
N1-β1
|
<1 (2)
|
<1 (2)
|
1.0 (2)
|
<1 (2)
|
<1 (1)
|
<1 (1)
|
<1 (1)
|
<1 (1)
|
N1-β2
|
<1 (3)
|
1.8 ± 0.6 (4)
|
1.2 ± 0.1 (3)
|
3.0 (2)
|
<1 (1)
|
<1 (1)
|
<1 (1)
|
<1 (1)
|
N1-α1
|
nd (1)
|
nd (1)
|
nd (1)
|
nd (1)
|
nd (1)
|
nd (1)
|
nd (1)
|
nd (1)
|
N1-α2
|
<1 (3)
|
7.0 ± 4.1 (5)
|
<1 (4)
|
46.8 ± 6.2 (4)
|
<1 (3)
|
40.1 ± 7.8 (4)
|
<1 (4)
|
56.1 ± 11.2 (4)
|
|
P < 0.01
|
P < 0.001
|
P < 0.001
|
P < 0.001
|
CKM
|
3 ± 2 (3)
|
1290 ± 296 (3)
|
3 ± 1 (4)
|
1492 ± 206 (3)
|
<1 (4)
|
2346 ± 768 (3)
|
2 ± 1 (4)
|
1279 ± 320 (4)
|
|
P < 0.005
|
P < 0.001
|
P < 0.005
|
P < 0.001
|
Desmin
|
10331 ± 671 (3)
|
13038 ± 303 (3)
|
4064 ± 673 (6)
|
14334 ± 581 (4)
|
6084 ± 160 (4)
|
16701 ± 1321 (4)
|
7952 ± 441 (4)
|
11358 ± 231 (4)
|
|
P < 0.005
|
P < 0.001
|
P < 0.001
|
P < 0.001
|
- Results expressed as: Mean ± SD (n), “n” = number of repeat measurements. nd = Not detected