scholarly journals Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Pauline Roy ◽  
Fredérique Rau ◽  
Julien Ochala ◽  
Julien Messéant ◽  
Bodvael Fraysse ◽  
...  
1985 ◽  
Vol 59 (1) ◽  
pp. 119-126 ◽  
Author(s):  
K. K. McCully ◽  
J. A. Faulkner

We tested the hypothesis that lengthening contractions result in greater injury to skeletal muscle fibers than isometric or shortening contractions. Mice were anesthetized with pentobarbital sodium and secured to a platform maintained at 37 degrees C. The distal tendon of the extensor digitorum longus muscle was attached to a servomotor. A protocol consisting of isometric, shortening, or lengthening contractions was performed. After the contraction protocol the distal tendon was reattached, incisions were closed, and the mice were allowed to recover. The muscles were removed after 1–30 days, and maximum isometric force (Po) was measured in vitro at 37 degrees C. Three days after isometric and shortening contractions and sham operations, histological appearance was not different from control and Po was 80% of the control value. Three days after lengthening contractions, histological sections showed that 37 +/- 4% of muscle fibers degenerated and Po was 22 +/- 3% of the control value. Muscle regeneration, first seen at 4 days, was nearly complete by 30 days, when Po was 84 +/- 3% of the control value. We conclude that, with the protocol used, lengthening, but not isometric or shortening contractions, caused significant injury to muscle fibers.


2009 ◽  
Vol 40 (4) ◽  
pp. 589-594 ◽  
Author(s):  
Richard M. Lovering ◽  
Alan B. McMillan ◽  
Rao P. Gullapalli

1998 ◽  
Vol 5 ◽  
pp. 125
Author(s):  
J. Komulainen ◽  
R. Kalliokoski ◽  
H. Kuipers ◽  
M.K.C. Hesselink

2016 ◽  
Vol 113 (39) ◽  
pp. 10992-10997 ◽  
Author(s):  
Erik P. Rader ◽  
Rolf Turk ◽  
Tobias Willer ◽  
Daniel Beltrán ◽  
Kei-ichiro Inamori ◽  
...  

Dystroglycan (DG) is a highly expressed extracellular matrix receptor that is linked to the cytoskeleton in skeletal muscle. DG is critical for the function of skeletal muscle, and muscle with primary defects in the expression and/or function of DG throughout development has many pathological features and a severe muscular dystrophy phenotype. In addition, reduction in DG at the sarcolemma is a common feature in muscle biopsies from patients with various types of muscular dystrophy. However, the consequence of disrupting DG in mature muscle is not known. Here, we investigated muscles of transgenic mice several months after genetic knockdown of DG at maturity. In our study, an increase in susceptibility to contraction-induced injury was the first pathological feature observed after the levels of DG at the sarcolemma were reduced. The contraction-induced injury was not accompanied by increased necrosis, excitation–contraction uncoupling, or fragility of the sarcolemma. Rather, disruption of the sarcomeric cytoskeleton was evident as reduced passive tension and decreased titin immunostaining. These results reveal a role for DG in maintaining the stability of the sarcomeric cytoskeleton during contraction and provide mechanistic insight into the cause of the reduction in strength that occurs in muscular dystrophy after lengthening contractions.


2016 ◽  
Vol 6 ◽  
Author(s):  
Michael R. Deyhle ◽  
Amanda M. Gier ◽  
Kaitlyn C. Evans ◽  
Dennis L. Eggett ◽  
W. Bradley Nelson ◽  
...  

1999 ◽  
Vol 276 (1) ◽  
pp. C120-C127 ◽  
Author(s):  
Keith Baar ◽  
Karyn Esser

High-resistance exercise training results in an increase in muscle wet mass and protein content. To begin to address the acute changes following a single bout of high-resistance exercise, a new model has been developed. Training rats twice a week for 6 wk resulted in 13.9 and 14.4% hypertrophy in the extensor digitorum longus (EDL) and tibialis anterior (TA) muscles, respectively. Polysome profiles after high-resistance lengthening contractions suggest that the rate of initiation is increased. The activity of the 70-kDa S6 protein kinase (p70S6k), a regulator of translation initiation, is also increased following high-resistance lengthening contractions (TA, 363 ± 29%; EDL, 353 ± 39%). Furthermore, the increase in p70S6kactivity 6 h after exercise correlates with the percent change in muscle mass after 6 wk of training ( r = 0.998). The tight correlation between the activation of p70S6kand the long-term increase in muscle mass suggests that p70S6kphosphorylation may be a good marker for the phenotypic changes that characterize muscle hypertrophy and may play a role in load-induced skeletal muscle growth.


2008 ◽  
Vol 17 (23) ◽  
pp. 3686-3696 ◽  
Author(s):  
Bert Blaauw ◽  
Cristina Mammucari ◽  
Luana Toniolo ◽  
Lisa Agatea ◽  
Reimar Abraham ◽  
...  

2018 ◽  
Vol 106 ◽  
pp. 28-38 ◽  
Author(s):  
Jacob R. Sorensen ◽  
Caitlin Skousen ◽  
Alex Holland ◽  
Kyle Williams ◽  
Robert D. Hyldahl

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