scholarly journals Old drug, new use: Can Amitriptyline improve mood and reduce skeletal muscle inflammation in a mouse model of Duchenne Muscular Dystrophy?

2012 ◽  
pp. 47-51
Author(s):  
Jennifer Manning

DMD is an inherited genetic disease affecting 1 in 3500 males. It results from the deletion of a gene which makes the protein Dystrophin. Dystrophin is essential for maintaining the structure of cells. In skeletal muscle, it acts as an anchor for structural components inside the cell. When dystrophin is missing, it leads to mechanical damage and tears in the muscle cell wall, which causes a cascade of events and leads to chronic inflammation. The chemical components of this inflammatory response lead to extensive muscle damage in sufferers. Muscle tissue is replaced with collagen and fat cells, leading to a significantly weaker and dysfunctional muscle. Sufferers of DMD are wheelchair-bound by their teenage years, and don’t normally live beyond their twenties. Clinical presentation of the disease occurs when young boys start walking; the weight put on the muscles of the calf exasperate the damage and they have difficulty running, jumping, ...

2019 ◽  
Vol 8 ◽  
pp. 204800401987958
Author(s):  
HR Spaulding ◽  
C Ballmann ◽  
JC Quindry ◽  
MB Hudson ◽  
JT Selsby

Background Duchenne muscular dystrophy is a muscle wasting disease caused by dystrophin gene mutations resulting in dysfunctional dystrophin protein. Autophagy, a proteolytic process, is impaired in dystrophic skeletal muscle though little is known about the effect of dystrophin deficiency on autophagy in cardiac muscle. We hypothesized that with disease progression autophagy would become increasingly dysfunctional based upon indirect autophagic markers. Methods Markers of autophagy were measured by western blot in 7-week-old and 17-month-old control (C57) and dystrophic (mdx) hearts. Results Counter to our hypothesis, markers of autophagy were similar between groups. Given these surprising results, two independent experiments were conducted using 14-month-old mdx mice or 10-month-old mdx/Utrn± mice, a more severe model of Duchenne muscular dystrophy. Data from these animals suggest increased autophagosome degradation. Conclusion Together these data suggest that autophagy is not impaired in the dystrophic myocardium as it is in dystrophic skeletal muscle and that disease progression and related injury is independent of autophagic dysfunction.


1995 ◽  
Vol 17 (3) ◽  
pp. 202-205 ◽  
Author(s):  
Hirotoshi Kinoshita ◽  
Yu-ichi Goto ◽  
Mitsuru Ishikawa ◽  
Tetsuya Uemura ◽  
Kouichi Matsumoto ◽  
...  

Nature ◽  
1988 ◽  
Vol 333 (6172) ◽  
pp. 466-469 ◽  
Author(s):  
Elizabeth E. Zubrzycka-Gaarn ◽  
Dennis E. Bulman ◽  
George Karpati ◽  
Arthur H. M. Burghes ◽  
Bonnie Belfall ◽  
...  

1980 ◽  
Vol 56 (2) ◽  
pp. 99-101 ◽  
Author(s):  
Kazuo MIYOSHI ◽  
Akira TAIRA ◽  
Kenzo YOSHIDA ◽  
Katsuya TAMURA ◽  
Shigetoshi UGA

2020 ◽  
Vol 11 (4) ◽  
pp. 1047-1069 ◽  
Author(s):  
Jessy Etienne ◽  
Pierre Joanne ◽  
Cyril Catelain ◽  
Stéphanie Riveron ◽  
Alexandra Clarissa Bayer ◽  
...  

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