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. 2008 Feb 1;118(2):445–453. doi: 10.1172/JCI34006

Figure 1. The RyR1 macromolecular complex.

Figure 1

RyR1 forms a macromolecular complex with PDE4D3, A-kinase anchor protein (mAKAP), the catalytic (cat) and regulatory (reg) subunits of PKA, calstabin1, calmodulin, PP1, and spinophilin. Components of the RyR1 complex are represented schematically bound to one monomer of the homotetrameric RyR1 channel. On the left, stress signals targeting RyR1 are shown, including the three hotspots on RyR1 where disease-causing mutations cluster (34, 114). MH-associated mutations are in red, CCD-associated mutations are in pink, mixed mutations or those associated with multi-minicore disease or nemaline rod disease are shown in black. –, deletion mutation.