scholarly journals Restriction of Calpain3 Expression to the Skeletal Muscle Prevents Cardiac Toxicity and Corrects Pathology in a Murine Model of Limb-Girdle Muscular Dystrophy

Circulation ◽  
2013 ◽  
Vol 128 (10) ◽  
pp. 1094-1104 ◽  
Author(s):  
Carinne Roudaut ◽  
Florence Le Roy ◽  
Laurence Suel ◽  
Jérôme Poupiot ◽  
Karine Charton ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Murine Model ◽  
Cardiac Toxicity ◽  
Limb Girdle Muscular Dystrophy

Pathophysiology of limb girdle muscular dystrophy type 2A: hypothesis and new insights into the IκBα/NF-κB survival pathway in skeletal muscle

2001 ◽  
Vol 79 (5-6) ◽  
pp. 254-261 ◽  
Author(s):  
Stephen Baghdiguian ◽  
Isabelle Richard ◽  
Marianne Martin ◽  
Peter Coopman ◽  
Jacques S. Beckmann ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Limb Girdle Muscular Dystrophy ◽  
Survival Pathway

scholarly journals The use of ivabradine in a patient with inappropriate sinus tachycardia and cardiomyopathy due to limb girdle muscular dystrophy type 2I

2020 ◽  
Vol 13 (1) ◽  
pp. e230647
Author(s):  
Rajkumar Rajendram ◽  
Fahad AlDhahri ◽  
Naveed Mahmood ◽  
Mubashar Kharal
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Cardiac Disease ◽  
Sinus Tachycardia ◽  
Prognostic Significance ◽  
Mechanisms Of Action ◽  
Limb Girdle Muscular Dystrophy ◽  
Muscular Dystrophies ◽  
Appropriate Use ◽  
Inappropriate Sinus Tachycardia

Muscular dystrophies are a heterogeneous group of disorders that commonly involve cardiac and skeletal muscle. Comprehensive guidelines for the management of cardiac failure and arrhythmias are available. However, the studies from which their recommendations are derived did not include any patients with muscular dystrophy. Some medications (eg, betablockers) may have significant side effects in this cohort. In some situations the use of agents with unique mechanisms of action such as ivabradine (a ‘funny’ channel inhibitor) may be more appropriate. Use of ivabradine has not previously been reported in limb girdle muscular dystrophy (LGMD). We describe the course of a patient with LGMD type 2I, cardiomyopathy and inappropriate sinus tachycardia treated with ivabradine. As advances in respiratory support have improved the outcomes of patients with muscular dystrophy; the prognostic significance of cardiac disease has increased. Ivabradine is tolerated and may reduce symptoms, morbidity and mortality in this cohort.

scholarly journals Age-Dependent Effect of Myostatin Blockade on Disease Severity in a Murine Model of Limb-Girdle Muscular Dystrophy

2006 ◽  
Vol 168 (6) ◽  
pp. 1975-1985 ◽  
Author(s):  
Stephanie A. Parsons ◽  
Douglas P. Millay ◽  
Michelle A. Sargent ◽  
Elizabeth M. McNally ◽  
Jeffery D. Molkentin
Keyword(s):  
Muscular Dystrophy ◽  
Disease Severity ◽  
Murine Model ◽  
Limb Girdle Muscular Dystrophy ◽  
Dependent Effect ◽  
Age Dependent

Magnetization Transfer Imaging of Skeletal Muscle in Autosomal Recessive Limb Girdle Muscular Dystrophy

1999 ◽  
Vol 23 (4) ◽  
pp. 609-614 ◽  
Author(s):  
James D. McDaniel ◽  
John L. Ulmer ◽  
Robert W. Prost ◽  
Malgorzata B. Franczak ◽  
Safwan Jaradeh ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Autosomal Recessive ◽  
Magnetization Transfer ◽  
Limb Girdle Muscular Dystrophy ◽  
Magnetization Transfer Imaging
Sign in / Sign up

Export Citation Format

Share Document