Effect of CAMK2D inhibition in RBM20 loss of function mouse models

Mutations in RBM20, a splicing factor that targets multiple pivotal cardiac genes including TTN and CAMK2D, cause a clinically aggressive form of dilated cardiomyopathy (DCM) with a high risk of malignant ventricular arrhythmias. We hypothesized that the RBM20 target CAMK2D contributes critically to RBM20 cardiomyopathy. Here, we sequenced the hearts of Rbm20/Camk2d double knockout mice, as well as the hearts of Rbm20-R636Q knock-in mice treated with the CAMK2 inhibitor Hesperadin. Overall design: RNAseq of RBM20/CAMK2D double knockout mice and RBM20-R636Q knock-in mice with CAMK2 inhibition using Hesperadin