MYH7 encodes myosin heavy chain 7, a sarcomeric protein essential for muscle contraction through its role as an actin-based motor protein with ATPase activity 1. The protein forms bipolar thick filaments that interact with actin thin filaments to generate force during the myosin power stroke, a process involving conformational changes in the converter domain and myosin mesa 2. MYH7 expression is developmentally regulated through multiple cardiac enhancers, including a key enhancer controlling the MYH6-to-MYH7 isoform switch 3. Genetic variants in MYH7 are among the most common causes of inherited cardiomyopathy, accounting for approximately 40% of familial hypertrophic cardiomyopathy (HCM) cases 4. Missense variants predominantly cause HCM through dominant-negative effects, with disease enrichment in functionally critical regions like the converter domain 2. Loss-of-function variants cause dilated cardiomyopathy (DCM), affecting 1-5% of DCM patients, characterized by progressive left ventricular dysfunction and early-onset disease with high phenotypic expression by age 60 5. Clinically, MYH7 variants also cause skeletal muscle myopathies and left ventricular non-compaction 1. The clinical severity and phenotypic expression of MYH7-related disease are modulated by regulatory variants in enhancer regions and variant location within the protein 36. Therapeutic approaches include gene editing and base editing to correct pathogenic variants, offering promise for precision treatment of HCM 7.