Key result
Genetic deletion of cMyBP-C in mouse cardiac myofibrils significantly reduced the overall proportion of myosin in the super-relaxed state from 57% to 35% compared to wildtype.
Why the study?
To define how MyBP-C and its specific domains contribute to stabilizing the super relaxed myosin state in cardiac muscle.
Absolute Event Rate: 35% vs 57%
p-value: p=<0.001
The N-terminus of cardiac MyBP-C is necessary for establishing the myosin Super Relaxed state along the entire thick filament, though an inherent structural bias also contributes to its stabilization.
cMyBP-C deletion disrupts myosin super-relaxed state in mice; leaves open its role in human cardiomyopathy.
The myosin Super Relaxed (SRX) state is central to striated muscle metabolic and functional regulation. In skeletal muscle, SRX myosin are predominantly colocalized with Myosin-Binding Protein C (MyBP-C) in the C-zone, proximal to the sarcomere center. To define how MyBP-C and its specific domains contribute to stabilizing the SRX state in cardiac muscle, we took advantage of transgenic MyBP-C null mice and those expressing MyBP-C with a 271 residue N-terminal truncation. Utilizing super-resolution microscopy, we determined the lifetime and sub-sarcomeric location of individual fluorescent-ATP turnover events within isolated cardiac myofibrils. The proportion of SRX myosin was highest in the C-zone (71±6%) and lower in the D-zone (45±10%) which lies farther from the sarcomere center and lacks MyBP-C, suggesting a possible role for MyBP-C in stabilizing the SRX state within the C-zone. However, myofibrils from MyBP-C null mice demonstrated an ~40% SRX reduction, not only within the now MyBP-C-free C-zone (49±9% SRX), but also within the D-zone (22±5% SRX). These data suggest that the influence of MyBP-C on the SRX state is not limited to the C-zone, but extends along the thick filament. Interestingly, myofibrils with N-terminal truncated MyBP-C had an SRX content and spatial gradient similar to the MyBP-C null, indicating that the N terminus of cardiac MyBP-C is necessary for MyBP-C’s role in establishing SRX along the entire thick filament. Given that SRX myosin are enriched in the C-zone, even in the absence of MyBP-C or its N-terminus, an inherent bias must exist in the structure of the thick filament to stabilize the SRX state. One candidate may be a differential in the super repeats of titin that interact with MyBP-C and myosin and which template the thick filament.
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Nelson et al. (2022) studied Cardiac muscle physiology (n=14). Genetic deletion of cMyBP-C vs. Wildtype was evaluated on Proportion of myosin in the Super Relaxed (SRX) state (p=<0.001). Genetic deletion of cMyBP-C in mouse cardiac myofibrils significantly reduced the overall proportion of myosin in the super-relaxed state from 57% to 35% compared to wildtype.
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