M.P.1.09 Mitochondrial disorders with isolated skeletal muscle defect

2007 ◽  
Vol 17 (9-10) ◽  
pp. 770
Author(s):  
C. Jardel ◽  
B. Eymard ◽  
I. Lemière ◽  
B. Hainque ◽  
A. Lombès ◽  
...  
2021 ◽  
Vol 43 (3) ◽  
pp. 1473-1488
Author(s):  
Seong-Eun Byun ◽  
Changgon Sim ◽  
Yoonhui Chung ◽  
Hyung Kyung Kim ◽  
Sungmoon Park ◽  
...  

Profound skeletal muscle loss can lead to severe disability and cosmetic deformities. Mesenchymal stem cell (MSC)-derived exosomes have shown potential as an effective therapeutic tool for tissue regeneration. This study aimed to determine the regenerative capacity of MSC-derived exosomes for skeletal muscle regeneration. Exosomes were isolated from human adipose tissue-derived MSCs (AD-MSCs). The effects of MSC-derived exosomes on satellite cells were investigated using cell viability, relevant genes, and protein analyses. Moreover, NOD-SCID mice were used and randomly assigned to the healthy control (n = 4), muscle defect (n = 6), and muscle defect + exosome (n = 6) groups. Muscle defects were created using a biopsy punch on the quadriceps of the hind limb. Four weeks after the surgery, the quadriceps muscles were harvested, weighed, and histologically analyzed. MSC-derived exosome treatment increased the proliferation and expression of myocyte-related genes, and immunofluorescence analysis for myogenin revealed a similar trend. Histologically, MSC-derived exosome-treated mice showed relatively preserved shapes and sizes of the muscle bundles. Immunohistochemical staining revealed greater expression of myogenin and myoblast determination protein 1 in the MSC-derived exosome-treated group. These results indicate that exosomes extracted from AD-MSCs have the therapeutic potential for skeletal muscle regeneration.


Mitochondrion ◽  
2004 ◽  
Vol 4 (5-6) ◽  
pp. 441-452 ◽  
Author(s):  
J.M. Bourgeois ◽  
M.A. Tarnopolsky

2008 ◽  
Vol 22 (S1) ◽  
Author(s):  
Edward K Merritt ◽  
Thomas J Walters ◽  
David G Baer ◽  
Roger P Farrar

2005 ◽  
Vol 19 (7) ◽  
pp. 1-30 ◽  
Author(s):  
Marco Crimi ◽  
Andreina Bordoni ◽  
Giorgia Menozzi ◽  
Laura Riva ◽  
Francesco Fortunate ◽  
...  

2015 ◽  
Vol 130 (3) ◽  
pp. 167-181 ◽  
Author(s):  
Valeria Guglielmi ◽  
Gaetano Vattemi ◽  
Roberto Chignola ◽  
Anna Chiarini ◽  
Matteo Marini ◽  
...  

We report the role and the relevance of programmed cell death (PCD) in patients with mitochondrial respiratory chain dysfunction. Our data throw light on to the pathogenesis of mitochondrial disorders and notably on to the fate of the affected muscle fibres


Author(s):  
D. E. Philpott ◽  
A. Takahashi

Two month, eight month and two year old rats were treated with 10 or 20 mg/kg of E. Coli endotoxin I. P. The eight month old rats proved most resistant to the endotoxin. During fixation the aorta, carotid artery, basil arartery of the brain, coronary vessels of the heart, inner surfaces of the heart chambers, heart and skeletal muscle, lung, liver, kidney, spleen, brain, retina, trachae, intestine, salivary gland, adrenal gland and gingiva were treated with ruthenium red or alcian blue to preserve the mucopolysaccharide (MPS) coating. Five, 8 and 24 hrs of endotoxin treatment produced increasingly marked capillary damage, disappearance of the MPS coating, edema, destruction of endothelial cells and damage to the basement membrane in the liver, kidney and lung.


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