Autonomic, locomotor and cardiac abnormalities in a mouse model of muscular dystrophy: targeting the renin-angiotensin system

Experimental Physiology
Rasna Sabharwal, Mark W Chapleau

Abstract

New Findings What is the topic of this review? This symposium report summarizes autonomic, cardiac and skeletal muscle abnormalities in sarcoglycan-δ-deficient mice (Sgcd-/-), a mouse model of limb girdle muscular dystrophy, with emphasis on the roles of autonomic dysregulation and activation of the renin-angiotensin system at a young age. What advances does it highlight? The contributions of the autonomic nervous system and the renin-angiotensin system to the pathogenesis of muscular dystrophy are highlighted. Results demonstrate that autonomic dysregulation precedes and predicts later development of cardiac dysfunction in Sgcd-/- mice and that treatment of young Sgcd-/- mice with the angiotensin type 1 receptor antagonist losartan or with angiotensin-(1-7) abrogates the autonomic dysregulation, attenuates skeletal muscle pathology and increases spontaneous locomotor activity. Muscular dystrophies are a heterogeneous group of genetic muscle diseases characterized by muscle weakness and atrophy. Mutations in sarcoglycans and other subunits of the dystrophin-glycoprotein complex cause muscular dystrophy and dilated cardiomyopathy in animals and humans. Aberrant autonomic signalling is recognized in a variety of neuromuscular dis...Continue Reading

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Citations

Oct 30, 2015·International Journal of Biological Macromolecules·Hui TaoJun Li
Apr 8, 2016·Hypertension Research : Official Journal of the Japanese Society of Hypertension·Daisy Motta-SantosMichael Bader
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Jan 6, 2021·International Journal of Molecular Sciences·Moises Rodriguez-GonzalezAna Castellano-Martinez
Jun 3, 2021·International Journal of Molecular Sciences·Daniela L RebolledoEnrique Brandan

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