The present invention refers to non-human transgenic mammals, preferably
rodents, or mice, which comprise a mutation in the gene encoding for the
cardiac ryanodine receptor (RyR2).Transgenic animals carrying the amino
acid change R4496C in the RyR2 protein show a phenotype similar to that
of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) (OMIM:
604772). Further provided are methods for using these animals as in vivo
model of Catecholaminergic Polymorphic Ventricular Tachycardia and RyR2
dependent arrhythmias, in drug screening and for understanding the
molecular basis of RyR2 dependent arrhythmias.