The role of cardiac myosin binding protein-C in familial hypertrophic cardiomyopathy

Cardiac myosin binding protein-C (cMyBP-C): C-terminal segment and its interaction with titin in healthy and diseased heart

Familial hypertrophic cardiomyopathy is a devastating inherited form of heart disease. In the young, it can lead to sudden death. Professor John Trinick from the University of Leeds has been awarded a 3-year research grant of £180,000 to study an important protein present in heart muscle called cardiac myosin binding protein-C (or cMyBP-C for short). Inherited genetic errors affecting this protein are thought to be responsible for familial hypertrophic cardiomyopathy, an inherited heart muscle disease. cMyBP-C is thought to be involved in the correct contraction of the heart muscle, and Prof Trinick will investigate how this protein is affected by genetic errors (called mutations). Using a powerful microscope (called an electron microscope), he will look for differences in the structure of mutated and normal cMyBP-C. These investigations will involve looking at how cMyBP-C interacts with other heart muscle proteins that are important for coordinated contraction. Identifying any differences will help to identify how any protein dysfunction could be corrected. Research is important for understanding the mechanisms that lead to this condition and how they can be treated in affected families.