The innate immune response comprised of DNA-sensing protein cyclic guanosine monophosphate–adenosine monophosphate synthase (CGAS) and its downstream molecules, the stimulator of interferon genes 1 (STING1), TANK-binding kinase 1 (TBK1), interferon regulatory factor 3 (IRF3), and nuclear factor kappa B (NFκB), are activated in several cardiovascular diseases, including hereditary cardiomyopathies, myocardial infarction, hypertension, atherosclerosis, and aortic aneurysm. Here, TBK1 is linked to familial cardiomyopathy.