Cardiac myosin-binding protein C (MYBPC3) in cardiac pathophysiology

L Carrier, G Mearini, K Stathopoulou, F Cuello - Gene, 2015 - Elsevier
More than 350 individual MYPBC3 mutations have been identified in patients with inherited
hypertrophic cardiomyopathy (HCM), thus representing 40–50% of all HCM mutations …

Cardiac myosin super relaxation (SRX): a perspective on fundamental biology, human disease and therapeutics

M Schmid, CN Toepfer - Biology open, 2021 - journals.biologists.com
The fundamental basis of muscle contraction 'the sliding filament model'(;) and the 'swinging,
tilting crossbridge-sliding filament mechanism'(;) nucleated a field of research that has …

Structure of the native myosin filament in the relaxed cardiac sarcomere

D Tamborrini, Z Wang, T Wagner, S Tacke, M Stabrin… - Nature, 2023 - nature.com
The thick filament is a key component of sarcomeres, the basic units of striated muscle.
Alterations in thick filament proteins are associated with familial hypertrophic …

[HTML][HTML] The molecular basis for sarcomere organization in vertebrate skeletal muscle

Z Wang, M Grange, T Wagner, AL Kho, M Gautel… - Cell, 2021 - cell.com
Sarcomeres are force-generating and load-bearing devices of muscles. A precise molecular
picture of how sarcomeres are built underpins understanding their role in health and …

Cryo-EM structure of the human cardiac myosin filament

D Dutta, V Nguyen, KS Campbell, R Padrón, R Craig - Nature, 2023 - nature.com
Pumping of the heart is powered by filaments of the motor protein myosin that pull on actin
filaments to generate cardiac contraction. In addition to myosin, the filaments contain cardiac …

Force generation by skeletal muscle is controlled by mechanosensing in myosin filaments

M Linari, E Brunello, M Reconditi, L Fusi, M Caremani… - Nature, 2015 - nature.com
Contraction of both skeletal muscle and the heart is thought to be controlled by a calcium-
dependent structural change in the actin-containing thin filaments, which permits the binding …

Effects of myosin variants on interacting-heads motif explain distinct hypertrophic and dilated cardiomyopathy phenotypes

L Alamo, JS Ware, A Pinto, RE Gillilan, JG Seidman… - elife, 2017 - elifesciences.org
Cardiac β-myosin variants cause hypertrophic (HCM) or dilated (DCM) cardiomyopathy by
disrupting sarcomere contraction and relaxation. The locations of variants on isolated …

Hypertrophic cardiomyopathy and the myosin mesa: viewing an old disease in a new light

DV Trivedi, AS Adhikari, SS Sarkar, KM Ruppel… - Biophysical …, 2018 - Springer
The sarcomere is an exquisitely designed apparatus that is capable of generating force,
which in the case of the heart results in the pumping of blood throughout the body. At the …

Titin force in muscle cells alters lattice order, thick and thin filament protein formation

AL Hessel, W Ma, N Mazara, PE Rice… - Proceedings of the …, 2022 - National Acad Sciences
Skeletal muscle force production is increased at longer compared to shorter muscle lengths
because of length-dependent priming of thick filament proteins in the contractile unit before …

Cardiac disorders and pathophysiology of sarcomeric proteins

J van der Velden, GJM Stienen - Physiological reviews, 2019 - journals.physiology.org
The sarcomeric proteins represent the structural building blocks of heart muscle, which are
essential for contraction and relaxation. During recent years, it has become evident that …