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PKA-phosphorylated CPVT variant human ryanodine receptor type 2 R2474S in the presence of Rycal ARM210 - RyR2-R2474S + ARM210

ABSTRACT:

SUBMITTER: Marco Cesar Miotto 

PROVIDER: EMPIAR-11402 | biostudies-other |

REPOSITORIES: biostudies-other

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Structural analyses of human ryanodine receptor type 2 channels reveal the mechanisms for sudden cardiac death and treatment.

Miotto Marco C MC   Weninger Gunnar G   Dridi Haikel H   Yuan Qi Q   Liu Yang Y   Wronska Anetta A   Melville Zephan Z   Sittenfeld Leah L   Reiken Steven S   Marks Andrew R AR  

Science advances 20220720 29

Ryanodine receptor type 2 (RyR2) mutations have been linked to an inherited form of exercise-induced sudden cardiac death called catecholaminergic polymorphic ventricular tachycardia (CPVT). CPVT results from stress-induced sarcoplasmic reticular Ca<sup>2+</sup> leak via the mutant RyR2 channels during diastole. We present atomic models of human wild-type (WT) RyR2 and the CPVT mutant RyR2-R2474S determined by cryo-electron microscopy with overall resolutions in the range of 2.6 to 3.6 Å, and re  ...[more]

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