Article
MYBPC3 mutations are associated with a reduced super-relaxed state in patients with hypertrophic cardiomyopathy.
PloS one - 1 Jan 2017
McNamara James W, Li Amy, Lal Sean, Bos J Martijn, Harris Samantha P, van der Velden Jolanda, Ackerman Michael J, Cooke Roger, Dos Remedios Cristobal G
Abstract excerpt
The "super-relaxed state" (SRX) of myosin represents a 'reserve' of motors in the heart. Myosin heads in the SRX are bound to the thick filament and have a very low ATPase rate. Changes in the SRX are likely to modulate cardiac contractility. We previously demonstrated that the SRX is significantly reduced in mouse cardiomyocytes lacking cardiac myosin binding protein-C (cMyBP-C). Here, we report the effect of...
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