scholarly journals Increasing taurine intake and taurine synthesis improves skeletal muscle function in the mdx mouse model for Duchenne muscular dystrophy

2016 ◽  
Vol 594 (11) ◽  
pp. 3095-3110 ◽  
Author(s):  
Jessica R. Terrill ◽  
Gavin J. Pinniger ◽  
Jamie A. Graves ◽  
Miranda D. Grounds ◽  
Peter G. Arthur
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Muscle Function ◽  
Mdx Mouse ◽  
Skeletal Muscle Function

scholarly journals Assessing the Use of the sGC Stimulator BAY-747, as a Potential Treatment for Duchenne Muscular Dystrophy

2021 ◽  
Vol 22 (15) ◽  
pp. 8016
Author(s):  
Shalini Murali Krishnan ◽  
Johannes Nordlohne ◽  
Lisa Dietz ◽  
Alexandros Vakalopoulos ◽  
Petra Haning ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Grip Strength ◽  
Muscle Function ◽  
Phase Iii ◽  
Potential Treatment ◽  
Skeletal Muscle Function ◽  
Therapeutic Benefits ◽  
Sgc Stimulators

Duchenne muscular dystrophy (DMD) is a severe and progressive muscle wasting disorder, affecting one in 3500 to 5000 boys worldwide. The NO-sGC-cGMP pathway plays an important role in skeletal muscle function, primarily by improving blood flow and oxygen supply to the muscles during exercise. In fact, PDE5 inhibitors have previously been investigated as a potential therapy for DMD, however, a large-scale Phase III clinical trial did not meet its primary endpoint. Since the efficacy of PDE5i is dependent on sufficient endogenous NO production, which might be impaired in DMD, we investigated if NO-independent sGC stimulators, could have therapeutic benefits in a mouse model of DMD. Male mdx/mTRG2 mice aged six weeks were given food supplemented with the sGC stimulator, BAY-747 (150 mg/kg of food) or food alone (untreated) ad libitum for 16 weeks. Untreated C57BL6/J mice were used as wild type (WT) controls. Assessments of the four-limb hang, grip strength, running wheel and serum creatine kinase (CK) levels showed that mdx/mTRG2 mice had significantly reduced skeletal muscle function and severe muscle damage compared to WT mice. Treatment with BAY-747 improved grip strength and running speed, and these mice also had reduced CK levels compared to untreated mdx/mTRG2 mice. We also observed increased inflammation and fibrosis in the skeletal muscle of mdx/mTRG2 mice compared to WT. While gene expression of pro-inflammatory cytokines and some pro-fibrotic markers in the skeletal muscle was reduced following BAY-747 treatment, there was no reduction in infiltration of myeloid immune cells nor collagen deposition. In conclusion, treatment with BAY-747 significantly improves several functional and pathological parameters of the skeletal muscle in mdx/mTRG2 mice. However, the effect size was moderate and therefore, more studies are needed to fully understand the potential treatment benefit of sGC stimulators in DMD.

scholarly journals Galectin‐3 and N ‐acetylglucosamine promote myogenesis and improve skeletal muscle function in the mdx model of Duchenne muscular dystrophy

2018 ◽  
Vol 32 (12) ◽  
pp. 6445-6455 ◽  
Author(s):  
Ann Rancourt ◽  
Sébastien S. Dufresne ◽  
Guillaume St‐Pierre ◽  
Julie‐Christine Lévesque ◽  
Haruka Nakamura ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Muscle Function ◽  
Skeletal Muscle Function ◽  
Galectin 3

scholarly journals Read-through compound 13 restores dystrophin expression and improves muscle function in the mdx mouse model for Duchenne muscular dystrophy

2012 ◽  
Vol 21 (18) ◽  
pp. 4007-4020 ◽  
Author(s):  
Refik Kayali ◽  
Jin-Mo Ku ◽  
Gregory Khitrov ◽  
Michael E. Jung ◽  
Olga Prikhodko ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mouse Model ◽  
Muscle Function ◽  
Mdx Mouse ◽  
Dystrophin Expression

scholarly journals Neopterin/7,8-dihydroneopterin is elevated in Duchenne muscular dystrophy patients and protects mdx skeletal muscle function

2018 ◽  
Vol 103 (7) ◽  
pp. 995-1009 ◽  
Author(s):  
Angus Lindsay ◽  
Alexandra Schmiechen ◽  
Christopher M. Chamberlain ◽  
James M. Ervasti ◽  
Dawn A. Lowe
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Muscle Function ◽  
Skeletal Muscle Function
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