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Mutations of the cardiac ryanodine receptor ( RyR2 ) gene in familial polymorphic ventricular tachycardia

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After mutation of the PAZ RNA-binding pocket (F294A, Y311A), trigger variants with longer internal loops induced binding and polyubiquitylation no better than did the seed-only variant, supporting the idea that together, trigger-specific RNA trajectory and binding of the vacated PAZ pocket substantially contribute to ZSWIM8 selectivity for TDMD substrates