Ryanodine Receptors are large ion channels responsible for the release of Ca2+ from the Endoplasmic and Sarcoplasmic Reticulum,a prerequisite for muscle contraction.Recent cryo-electron microscopy data have allowed a direct visualization of allosteric motions within these membrane protein giants.
The human genome encodes three Ryanodine Receptors (RyR) isoforms consisting of~ 5 000 amino acid subunits that assemble into > 2.2 MDa homotetramers.RyR1 is the primary skeletal muscle isoform and is the target for hundreds of mutations linked to central core disease and malignant hyperthermia.RyR2 predominates in the heart and is linked to stress-induced cardiac arrhythmias [1].