title: [Ryanodine receptor and heart disease].
abstract: Calcium ions (Ca2+) play an essential role in cardiac excitation-contraction coupling. Ca2+ is stored in the sarcoplasmic reticulum (SR) and is release via SR-Ca-release channels (ryanodine receptors, RyR2) to trigger contraction. RyR2 is a homotetramer comprising 4 pore-forming subunits. Each subunit is closely associated to regulatory proteins such as calstabine 2 (FKBP12.6), calmodulin, PKA, CamKII, calsequestrin and form a macromolecular complex that plays a critical role in pathological conditions. As a matter of fact, alterations of the channel activity and/or associated regulatory proteins can cause severe functional alterations resulting in arrhythmias and sudden death. Thus, RyR2 represent a novel therapeutic target and the discovery of a new pharmacological agent able to restore a normal RyR2 channel function represents a major challenge in the cardiac field.
authors.affiliation: Inserm, U 637, F-34925 Montpellier, France. alain.lacampagne@inserm.fr
authors.forename: Alain
authors.initials: A
authors.lastname: Lacampagne
authors.forename: Jérémy
authors.initials: J
authors.lastname: Fauconnier
authors.forename: Sylvain
authors.initials: S
authors.lastname: Richard
mesh_terms: D002462:Cell Membrane / Q000502:physiology; D006331:Heart Diseases / Q000503:physiopathology*; D006339:Heart Rate; D006706:Homeostasis; D006801:Humans; D019837:Ryanodine Receptor Calcium Release Channel / Q000502:physiology*; D017180:Tachycardia, Ventricular / Q000503:physiopathology
chemical_list: D019837:Ryanodine Receptor Calcium Release Channel