Ryanodine Receptors: Structure and Function

Overview

Journal J Biol Chem

Specialty Biochemistry

Date 2012 Jul 24

PMID 22822064

Citations 103

Authors

Filip Van Petegem

Affiliations

Soon will be listed here.

Abstract

Ryanodine receptors (RyRs) are huge ion channels that are responsible for the release of Ca(2+) from the sarco/endoplasmic reticulum. RyRs form homotetramers with a mushroom-like shape, consisting of a large cytoplasmic head and transmembrane stalk. Ca(2+) is a major physiological ligand that triggers opening of RyRs, but a plethora of modulatory proteins and small molecules in the cytoplasm and sarco/endoplasmic reticulum lumen have been recognized. Over 300 mutations in RyRs are associated with severe skeletal muscle disorders or triggered cardiac arrhythmias. With the advent of high-resolution structures of individual domains, many of these can be mapped onto the three-dimensional structure.

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