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6 Results Found

  • Review
  • Open Access
17 Citations
4,612 Views
19 Pages

23 October 2018

Mutations in the gene encoding for the intracellular protein dystrophin cause severe forms of muscular dystrophy. These so-called dystrophinopathies are characterized by skeletal muscle weakness and degeneration. Dystrophin deficiency also gives rise...

  • Review
  • Open Access
11 Citations
4,616 Views
23 Pages

Role of the Renin–Angiotensin–Aldosterone System in Dystrophin-Deficient Cardiomyopathy

  • Moises Rodriguez-Gonzalez,
  • Manuel Lubian-Gutierrez,
  • Helena Maria Cascales-Poyatos,
  • Alvaro Antonio Perez-Reviriego and
  • Ana Castellano-Martinez

31 December 2020

Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to revie...

  • Review
  • Open Access
22 Citations
5,790 Views
12 Pages

5 December 2019

Duchenne and Becker muscular dystrophies (DMD/BMD) result in progressive weakness of skeletal and cardiac muscles due to the deficiency of functional dystrophin. Respiratory failure is a leading cause of mortality in DMD patients; however, improved m...

  • Article
  • Open Access
1 Citations
5,593 Views
20 Pages

Dystrophin Deficiency Causes Progressive Depletion of Cardiovascular Progenitor Cells in the Heart

  • Sarka Jelinkova,
  • Yvonne Sleiman,
  • Petr Fojtík,
  • Franck Aimond,
  • Amanda Finan,
  • Gerald Hugon,
  • Valerie Scheuermann,
  • Deborah Beckerová,
  • Olivier Cazorla and
  • Albano C. Meli
  • + 8 authors

Duchenne muscular dystrophy (DMD) is a devastating condition shortening the lifespan of young men. DMD patients suffer from age-related dilated cardiomyopathy (DCM) that leads to heart failure. Several molecular mechanisms leading to cardiomyocyte de...

  • Article
  • Open Access
11 Citations
3,700 Views
19 Pages

RANKL Inhibition Reduces Cardiac Hypertrophy in mdx Mice and Possibly in Children with Duchenne Muscular Dystrophy

  • Laetitia Marcadet,
  • Emma Sara Juracic,
  • Nasrin Khan,
  • Zineb Bouredji,
  • Hideo Yagita,
  • Leanne M. Ward,
  • A. Russell Tupling,
  • Anteneh Argaw and
  • Jérôme Frenette

3 June 2023

Cardiomyopathy has become one of the leading causes of death in patients with Duchenne muscular dystrophy (DMD). We recently reported that the inhibition of the interaction between the receptor activator of nuclear factor κB ligand (RANKL) and...

  • Article
  • Open Access
24 Citations
6,325 Views
16 Pages

Tempol Supplementation Restores Diaphragm Force and Metabolic Enzyme Activities in mdx Mice

  • David P. Burns,
  • Izza Ali,
  • Clement Rieux,
  • James Healy,
  • Greg Jasionek and
  • Ken D. O’Halloran

6 December 2017

Duchenne muscular dystrophy (DMD) is characterized by striated muscle weakness, cardiomyopathy, and respiratory failure. Since oxidative stress is recognized as a secondary pathology in DMD, the efficacy of antioxidant intervention, using the superox...