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Vakrou S, Abraham MR. Hypertrophic cardiomyopathy: a heart in need of an energy bar?. Front Physiol. 2014;5:309doi: 10.3389/fphys.2014.00309.
Vakrou, S., & Abraham, M. R. (2014). Hypertrophic cardiomyopathy: a heart in need of an energy bar?. Frontiers in physiology, 5309. https://doi.org/10.3389/fphys.2014.00309
Vakrou, Styliani, and Abraham, M Roselle. "Hypertrophic cardiomyopathy: a heart in need of an energy bar?." Frontiers in physiology vol. 5 (2014): 309. doi: https://doi.org/10.3389/fphys.2014.00309
Vakrou S, Abraham MR. Hypertrophic cardiomyopathy: a heart in need of an energy bar?. Front Physiol. 2014 Aug 19;5:309. doi: 10.3389/fphys.2014.00309. eCollection 2014. PMID: 25191275; PMCID: PMC4137386.
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Front Physiol. 2014 Aug 19;5:309. doi: 10.3389/fphys.2014.00309. eCollection 2014.

Hypertrophic cardiomyopathy: a heart in need of an energy bar?.

Frontiers in physiology

Styliani Vakrou, M Roselle Abraham

Affiliations

  1. Division of Cardiology, School of Medicine, Johns Hopkins University Baltimore, MD, USA.

PMID: 25191275 PMCID: PMC4137386 DOI: 10.3389/fphys.2014.00309

Abstract

Hypertrophic cardiomyopathy (HCM) has been recently recognized as the most common inherited cardiovascular disorder, affecting 1 in 500 adults worldwide. HCM is characterized by myocyte hypertrophy resulting in thickening of the ventricular wall, myocyte disarray, interstitial and/or replacement fibrosis, decreased ventricular cavity volume and diastolic dysfunction. HCM is also the most common cause of sudden death in the young. A large proportion of patients diagnosed with HCM have mutations in sarcomeric proteins. However, it is unclear how these mutations lead to the cardiac phenotype, which is variable even in patients carrying the same causal mutation. Abnormalities in calcium cycling, oxidative stress, mitochondrial dysfunction and energetic deficiency have been described constituting the basis of therapies in experimental models of HCM and HCM patients. This review focuses on evidence supporting the role of cellular metabolism and mitochondria in HCM.

Keywords: bioenergetic deficit; calcium handling; hypertrophic cardiomyopathy; induced pluripotent stem cells (iPSCs); mitochondria

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