Cardiac myosin-binding protein C (MYBPC3) in cardiac pathophysiology
نویسندگان
چکیده
منابع مشابه
Cardiac myosin binding protein C.
Myosin binding protein C (MyBP-C) is one of a group of myosin binding proteins that are present in the myofibrils of all striated muscle. The protein is found at 43-nm repeats along 7 to 9 transverse lines in a portion of the A band where crossbridges are found (C zone). MyBP-C contains myosin and titin binding sites at the C terminus of the molecule in all 3 of the isoforms (slow skeletal, fas...
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The role of cardiac myosin binding protein-C (cMyBP-C) phosphorylation in cardiac physiology or pathophysiology is unclear. To investigate the status of cMyBP-C phosphorylation in vivo, we determined its phosphorylation state in stressed and unstressed mouse hearts. cMyBP-C phosphorylation is significantly decreased during the development of heart failure or pathologic hypertrophy. We then gene...
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Familial hypertrophic cardiomyopathy (FHC) is an inherited autosomal dominant disease caused by mutations in sarcomeric proteins. Among these, mutations that affect myosin binding protein-C (MyBP-C), an abundant component of the thick filaments, account for 20% to 30% of all mutations linked to FHC. However, the mechanisms by which MyBP-C mutations cause disease and the function of MyBP-C are n...
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Homozygous cardiac myosin binding protein C-deficient (Mybpc(t/t)) mice develop dramatic cardiac dilation shortly after birth; heart size increases almost twofold. We have investigated the mechanism of cardiac enlargement in these hearts. Throughout embryogenesis myocytes undergo cell division while maintaining the capacity to pump blood by rapidly disassembling and reforming myofibrillar compo...
متن کاملMyosin binding protein C, a potential regulator of cardiac contractility.
The role of myosin binding protein C (MyBP-C) in the formation and function of striated muscle is unclear, even though the demonstration of its presence in the thick filaments of striated muscle was made by Offer et al1 more than 25 years ago.2 Because of the inability of myosin to form normal thick filaments in the absence of MyBP-C and the temporal correlation between the appearance of MyBP-C...
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ژورنال
عنوان ژورنال: Gene
سال: 2015
ISSN: 0378-1119
DOI: 10.1016/j.gene.2015.09.008