The human plakoglobin cDNA with a 2 bp deletion at nucleotides 2157 and 2158 is under the control of the cardiac-specific mouse alpha-myosin heavy chain promoter. This 2 bp deletion is seen in individuals with Naxos disease. Four lines were generated but only one founder line was propagated in which expression of the exogenous truncated protein is at roughly equivalent levels to expression of the wild-type endogenous mouse protein.