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Arch Biochem Biophys. 2018 Dec 15;660:121-128. doi: 10.1016/j.abb.2018.10.007. Epub 2018 Oct 17.

Skeletal myosin binding protein-C: An increasingly important regulator of striated muscle physiology.

Author information

1
Heart, Lung and Vascular Institute, Department of Internal Medicine, Division of Cardiovascular Health and Disease, University of Cincinnati, Cincinnati, OH 45236, USA.
2
Heart, Lung and Vascular Institute, Department of Internal Medicine, Division of Cardiovascular Health and Disease, University of Cincinnati, Cincinnati, OH 45236, USA. Electronic address: sadayasl@ucmail.uc.edu.

Abstract

The Myosin Binding Protein-C (MyBP-C) family is a group of sarcomeric proteins important for striated muscle structure and function. Comprising approximately 2% of the myofilament mass, MyBP-C has important roles in both contraction and relaxation. Three paralogs of MyBP-C are encoded by separate genes with distinct expression profiles in striated muscle. In mammals, cardiac MyBP-C is limited to the heart, and it is the most extensively studied owing to its involvement in cardiomyopathies. However, the roles of two skeletal paralogs, slow and fast, in muscle biology remain poorly characterized. Nonetheless, both have been recently implicated in the development of skeletal myopathies. This calls for a better understanding of their function in the pathophysiology of distal arthrogryposis. This review characterizes MyBP-C as a whole and points out knowledge gaps that still remain with respect to skeletal MyBP-C.

KEYWORDS:

Distal arthrogryposis; MYBPC; Myofilament; Sarcomere; Striated muscle

PMID:
30339776
PMCID:
PMC6289839
[Available on 2019-12-15]
DOI:
10.1016/j.abb.2018.10.007

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