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2v6h
From Proteopedia
| 2v6h, resolution 1.55Å () | |||||||||
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| Related: | 1gxe, 1pd6 | ||||||||
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| Resources: | FirstGlance, OCA, PDBsum, RCSB | ||||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||||
CRYSTAL STRUCTURE OF THE C1 DOMAIN OF CARDIAC MYOSIN BINDING PROTEIN-C
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.
Crystal structure of the C1 domain of cardiac myosin binding protein-C: implications for hypertrophic cardiomyopathy., Govada L, Carpenter L, da Fonseca PC, Helliwell JR, Rizkallah P, Flashman E, Chayen NE, Redwood C, Squire JM, J Mol Biol. 2008 Apr 25;378(2):387-97. Epub 2008 Mar 4. PMID:18374358
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
About this Structure
2V6H is a 1 chain structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Govada L, Carpenter L, da Fonseca PC, Helliwell JR, Rizkallah P, Flashman E, Chayen NE, Redwood C, Squire JM. Crystal structure of the C1 domain of cardiac myosin binding protein-C: implications for hypertrophic cardiomyopathy. J Mol Biol. 2008 Apr 25;378(2):387-97. Epub 2008 Mar 4. PMID:18374358 doi:10.1016/j.jmb.2008.02.044
Page seeded by OCA on Tue Feb 17 17:19:33 2009
Categories: Homo sapiens | Carpenter, L. | Chayen, N E. | Flashman, E. | Fonseca, P C.A Da. | Govata, L. | Helliwell, J R. | Redwood, C. | Rizkallah, P J. | Squire, J M. | Actin-binding | Cardiomyopathy | Cell adhesion | Disease mutation | Hypertropic cardiomyopathy | Igi domain structure | Immunoglobulin domain | Muscle protein | Muscle regulation | Mybp-c c1 domain | Phosphorylation | Polymorphism | Thick filament
