N-terminal extension in cardiac myosin-binding protein C regulates myofilament binding.

ABSTRACT:

Rationale

Mutations in the gene encoding the sarcomeric protein cardiac myosin-binding protein C (cMyBP-C) are a leading cause of hypertrophic cardiomyopathy (HCM). Mouse models targeting cMyBP-C and use of recombinant proteins have been effective in studying its roles in contractile function and disease. Surprisingly, while the N-terminus of cMyBP-C is important to regulate myofilament binding and contains many HCM mutations, an incorrect sequence, lacking the N-terminal 8 amino acids has been used in many studies.

Objectives

To determine the N-terminal cMyBP-C sequences in ventricles and investigate the roles of species-specific differences in cMyBP-C on myofilament binding.

Methods and results

We determined cMyBP-C sequences in mouse and human by inspecting availab

SUBMITTER: Bunch TA 

PROVIDER: S-EPMC6366860 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature