title: Calcium Ions in Inherited Cardiomyopathies.
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 Ca(2+) 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 Ca(2+) 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 Ca(2+) sensitivity, together with the results of attempts to reverse the manifestation of heart failure by manipulating Ca(2+) homeostasis.
authors.forename: Spyridon
authors.initials: S
authors.lastname: Deftereos
authors.affiliation: Department of Cardiology, Athens General Hospital "G. Gennimatas", Mesogeion 154, 11527, Athens, Greece. npapouts2@gmail.com.
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Papoutsidakis
authors.forename: Georgios
authors.initials: G
authors.lastname: Giannopoulos
authors.forename: Christos
authors.initials: C
authors.lastname: Angelidis
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Raisakis
authors.forename: Georgios
authors.initials: G
authors.lastname: Bouras
authors.forename: Periklis
authors.initials: P
authors.lastname: Davlouros
authors.forename: Vasiliki
authors.initials: V
authors.lastname: Panagopoulou
authors.forename: John
authors.initials: J
authors.lastname: Goudevenos
authors.forename: Michael W
authors.initials: MW
authors.lastname: Cleman
authors.forename: John
authors.initials: J
authors.lastname: Lekakis
mesh_terms: D000818:Animals; D017209:Apoptosis; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D002118:Calcium / Q000378:metabolism*; D002135:Calcium-Binding Proteins / Q000235:genetics / Q000378:metabolism; D009202:Cardiomyopathies / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D002413:Cations, Divalent; D006330:Heart Defects, Congenital / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D006801:Humans; D014335:Tropomyosin / Q000235:genetics / Q000378:metabolism; D014336:Troponin / Q000235:genetics / Q000378:metabolism; D000099211:Phospholamban
chemical_list: D002118:Calcium; D002135:Calcium-Binding Proteins; D002413:Cations, Divalent; D014335:Tropomyosin; D014336:Troponin; D000099211:Phospholamban