Titin (TTN) plays essential roles in both skeletal and cardiac muscle and when functioning improperly has devastating effects on muscle like dilated cardiomyopathy. Gene therapy experts at Nationwide Children’s Hospital have utilized CRISPR/Cas9 technology to develop a new mouse model of dilated cardiomyopathy referred to as TTN326. Utilizing the CRISPR/Cas9 technology to produce a mouse model instead of traditional methods has reduced the time required to modify the Titin gene as well as off-target insertions into the mouse genome. Our experts have demonstrated functional deficits in skeletal muscles of the TTN-326 mouse model and plan to test therapeutic strategies intramuscularly and systemically in this model to restore Titin protein function.