It is not clear how the mutant truncated titin protein malfunctions or how it results in cardiac remodeling39; however, recent studies demonstrate that antisense-mediated exon skipping in human and mouse models of DCM carrying the frameshift mutation in titin exon 326 can improve myofibril assembly and function in homozygous embryos and rescue the DCM phenotype in heterozygous animals40. This evidence concerns the gene TTN and familial dilated cardiomyopathy.