motifs/domains
| four N-terminal regulatory domains, C0-C1-m-C2 (C0C2), influencing actin and myosin interactions, the basic contractile proteins of muscle |
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a cardiac-specific Ig-like domain C0, interacting with the regulatory light chain of myosin, thus placing the N terminus of the protein in proximity to the motor domain of myosin |
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C0 and C1 domain can each bind to the same two distinctly different positions on F-actin |
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seven immunoglobulin Ig C2 domains |
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third immunoglobulin domain of the cardiac isoform (cC2), having the beta-sandwich structure expected from a member of the immunoglobulin fold, and so-called "motif," located between the second and third immunoglobulin modules of the cardiac isoform, that is required for its Ca2+-dependent interaction with calmodulin |
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a M-domain, the major regulatory subunit of cardiac myosin-binding protein-C (MYBPC3) that modulates actin and myosin interactions to influence muscle contraction |
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three fibronectin, type III domains |
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two myosin binding sites, one close to the N terminus and the other at the C terminus |
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C terminal region binds to myosin rods and stabilizes thick filament structure |
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basic FUNCTION
| acting as a cross-bridge between myosin and titin, mediating adrenergic stimulation of cardiac contraction |
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accessory protein of striated muscle sarcomeres that is vital for maintaining regular heart function |
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multidomain protein present in the thick filaments of striated muscles and is involved in both sarcomere formation and contraction regulation |
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critical nodal point that has both important structural and signaling roles and whose modifications are known to cause significant human cardiac disease |
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myofibrillar protein important for normal myocardial contractility and stability |
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functions likely as a physiological brake on contraction by positioning myosin heads away from the thin filament, a constraint which is removed upon adrenergic stimulation or MYBPC3 ablation |
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multi-domain (C0-C10) protein that regulates heart muscle contraction through interaction with myosin, actin and other sarcomeric protein |
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role of LRP1 in the regulation and turnover of synaptic proteins, which may contribute to the reduced dendritic branching and to the neurological phenotype observed in the absence of LRP1 |
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is dispensable for the development of skeletal muscle with no functional or structural consequences in the adult myocyte, and skeletal isoforms can transcomplement in the heart in the absence of MYBPC3 |
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activates thin filaments and inhibits thick filaments in heart muscle cells |
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thick filament-associated protein that seems to contribute to the regulation of cardiac contraction through interactions with either myosin or actin or both |
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regulates the rate and force of contraction in mammalian myocardium |
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signaling node in cardiac myocytes that contributes to the maintenance of sarcomeric structure and regulation of contraction and relaxation |
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is a potent modulator of cardiac contractility |
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intrinsic role of MYBPC3 in maintaining cardiac fibroblast homeostasis and disruption of MYBPC3 in these cells contributes to the disease pathogenesis of hypertrophic cardiomyopathy (HCM) |