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C-protein is a major component of skeletal and cardiac muscle thick filaments. Mutations in the gene encoding cardiac C-protein [cardiac myosin binding protein-C (cMyBP-C)] are one of the principal causes of hypertrophic cardiomyopathy. cMyBP-C is a string of globular domains including eight immunoglobulin-like and three fibronectin-like domains termed C0-C10. It binds to myosin and titin, and probably to actin, and may have both a structural and a regulatory role in muscle function. To help to understand the pathology of the known mutations, we have solved the structure of the immunoglobulin-like C1 domain of MyBP-C by X-ray crystallography to a resolution of 1.55 A. Mutations associated with hypertrophic cardiomyopathy are clustered at one end towards the C-terminus, close to the important C1C2 linker, where they alter the structural integrity of this region and its interactions.

Original publication

DOI

10.1016/j.jmb.2008.02.044

Type

Journal article

Journal

J Mol Biol

Publication Date

25/04/2008

Volume

378

Pages

387 - 397

Keywords

Amino Acid Sequence, Cardiomyopathy, Hypertrophic, Carrier Proteins, Crystallography, X-Ray, Humans, Molecular Sequence Data, Mutation, Protein Folding, Protein Structure, Tertiary