Calcium Ions in Inherited Cardiomyopathies

Title:Calcium Ions in Inherited Cardiomyopathies

Volume: 12 Issue: 2

Author(s): Spyridon Deftereos, Nikolaos Papoutsidakis, Georgios Giannopoulos, Christos Angelidis, Konstantinos Raisakis, Georgios Bouras, Periklis Davlouros, Vasiliki Panagopoulou, John Goudevenos, Michael W. Cleman and John Lekakis

Affiliation:

Keywords: calcium, cardiomyopathy, Ca²⁺ sensitivity, hypertrophic, dilated, restrictive.

Abstract: Inherited cardiomyopathies are a known cause of heart failure, although the pathways and mechanisms leading from mutation to the heart failure phenotype have not been elucidated. There is strong evidence that this transition is mediated, at least in part, by abnormal intracellular Ca²⁺ handling, a key ion in ventricular excitation, contraction and relaxation. Studies in human myocytes, animal models and in vitro reconstituted contractile protein complexes have shown consistent correlations between Ca²⁺ sensitivity and cardiomyopathy phenotype, irrespective of the causal mutation. In this review we present the available data about the connection between mutations linked to familial hypertrophic (HCM), dilated (DCM) and restrictive (RCM) cardiomyopathy, right ventricular arrhythmogenic cardiomyopathy/dysplasia (ARVC/D) as well as left ventricular non-compaction and the increase or decrease in Ca²⁺ sensitivity, together with the results of attempts to reverse the manifestation of heart failure by manipulating Ca²⁺ homeostasis.

Cite this article as:

Deftereos Spyridon, Papoutsidakis Nikolaos, Giannopoulos Georgios, Angelidis Christos, Raisakis Konstantinos, Bouras Georgios, Davlouros Periklis, Panagopoulou Vasiliki, Goudevenos John, Cleman W. Michael and Lekakis John, Calcium Ions in Inherited Cardiomyopathies, Medicinal Chemistry 2016; 12 (2) . https://dx.doi.org/10.2174/157340641202160209093713