Ryanodine receptors (RyRs) are huge homotetrameric Ca2+ release channels localized to the sarcoplasmic reticulum. RyRs are responsible for the release of Ca2+ from the SR during excitation-contraction coupling in striated muscle cells. Recent revolutionary advancements in cryo-electron microscopy have provided a number of near-atomic structures of RyRs, which have enabled us to better understand the architecture of RyRs. Thus, we are now in a new era understanding the gating, regulatory and disease-causing mechanisms of RyRs. Here we review recent advances in the elucidation of the structures of RyRs, especially RyR1 in skeletal muscle, and their mechanisms of regulation by small molecules, associated proteins and disease-causing mutations.

中文翻译：

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结构研究的最新进展揭示了兰尼碱受体/Ca2+ 释放通道的调控机制。

Ryanodine 受体 (RyRs) 是位于肌浆网的巨大同型四聚体 Ca2+ 释放通道。RyRs 负责在横纹肌细胞的兴奋-收缩耦合过程中从 SR 释放 Ca2+。最近低温电子显微镜的革命性进展提供了许多 RyRs 的近原子结构，这使我们能够更好地了解 RyRs 的结构。因此，我们现在处于了解 RyRs 的门控、调节和致病机制的新时代。在这里，我们回顾了阐明 RyRs 结构的最新进展，尤其是骨骼肌中的 RyR1，以及它们通过小分子、相关蛋白和致病突变的调节机制。