CS tissue from SUR2[R1154Q] mice and human induced pluripotent stem cell–derived (hiPSC-derived) cardiomyocytes showed only full-length SUR2 transcripts, although further studies will be required in order to fully test whether SUR2[R1154Q] or other CS mutations might result in aberrant splicing and variable expressivity of disease features in human CS. The gene discussed is ABCC9; the disease is Cowden syndrome 1.