How does the MyBP-C protein cause cardiomyopathy?

Myosin Binding Protein-C: a key regulator of cardiac output

BHF Intermediate Basic Science Research Fellow Dr Thomas Kampourakis is studying how faulty proteins lead to cardiomyopathies, diseases of the heart muscle that can cause heart failure. When the heart beats many elements work together to do specific jobs at specific times. The smallest building block of the heart muscle is the sarcomere, and proteins that form part of the sarcomere are important for the heart to contract and beat properly. Faults in a sarcomere protein called myosin binding protein-C (MyBP-C) can cause cardiomyopathies, including dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM), and are a common cause of heart failure. Understanding what MyBP-C does when the heart beats may help scientists design better treatments for people with cardiomyopathy. In this project, Dr Kampourakis will study how MyBP-C helps to coordinate heart muscle contraction at the molecular level. He will manipulate portions of the MyBP-C protein (called fragments) in heart muscle cells and study how the properties of each fragment affect how the cells work. He will find out what controls MyBP-C and how faults in MyBP-C could cause hypertrophic cardiomyopathy. Understanding more about the MyBP-C protein could reveal a new way to treat cardiomyopathy, and pave the way for clinical trials to test new drugs that target it.