Mavacamten, a precision medicine for hypertrophic cardiomyopathy: From a motor protein to patients
Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder characterized by left
ventricular hypertrophy, hyperdynamic contraction, and impaired relaxation of the heart …
ventricular hypertrophy, hyperdynamic contraction, and impaired relaxation of the heart …
[HTML][HTML] Regulating striated muscle contraction: Through thick and thin
E Brunello, L Fusi - Annual Review of Physiology, 2024 - annualreviews.org
Force generation in striated muscle is primarily controlled by structural changes in the actin-
containing thin filaments triggered by an increase in intracellular calcium concentration …
containing thin filaments triggered by an increase in intracellular calcium concentration …
[HTML][HTML] Small angle X-ray diffraction as a tool for structural characterization of muscle disease
Small angle X-ray fiber diffraction is the method of choice for obtaining molecular level
structural information from striated muscle fibers under hydrated physiological conditions …
structural information from striated muscle fibers under hydrated physiological conditions …
[HTML][HTML] Hypertrophic cardiomyopathy: mutations to mechanisms to therapies
M Kawana, JA Spudich, KM Ruppel - Frontiers in Physiology, 2022 - frontiersin.org
Hypertrophic cardiomyopathy (HCM) affects more than 1 in 500 people in the general
population with an extensive burden of morbidity in the form of arrhythmia, heart failure, and …
population with an extensive burden of morbidity in the form of arrhythmia, heart failure, and …
A Laing distal myopathy–associated proline substitution in the β-myosin rod perturbs myosin cross-bridging activity
M Buvoli, GCK Wilson, A Buvoli… - Journal of Clinical …, 2024 - Am Soc Clin Investig
Proline substitutions within the coiled-coil rod region of the β-myosin gene (MYH7) are the
predominant mutations causing Laing distal myopathy (MPD1), an autosomal dominant …
predominant mutations causing Laing distal myopathy (MPD1), an autosomal dominant …
Myosin in autoinhibited off state(s), stabilized by mavacamten, can be recruited in response to inotropic interventions
W Ma, CL Del Rio, L Qi, M Prodanovic… - Proceedings of the …, 2024 - National Acad Sciences
Mavacamten is a FDA-approved small-molecule therapeutic designed to regulate cardiac
function at the sarcomere level by selectively but reversibly inhibiting the enzymatic activity …
function at the sarcomere level by selectively but reversibly inhibiting the enzymatic activity …
Danicamtiv increases myosin recruitment and alters cross-bridge cycling in cardiac muscle
BACKGROUND: Modulating myosin function is a novel therapeutic approach in patients with
cardiomyopathy. Danicamtiv is a novel myosin activator with promising preclinical data that …
cardiomyopathy. Danicamtiv is a novel myosin activator with promising preclinical data that …
Functional control of myosin motors in the cardiac cycle
M Irving - Nature Reviews Cardiology, 2024 - nature.com
Contraction of the heart is driven by cyclical interactions between myosin and actin filaments
powered by ATP hydrolysis. The modular structure of heart muscle and the organ-level …
powered by ATP hydrolysis. The modular structure of heart muscle and the organ-level …
[HTML][HTML] The biochemically defined super relaxed state of myosin—a paradox
The biochemical SRX (super-relaxed) state of myosin has been defined as a low ATPase
activity state. This state can conserve energy when the myosin is not recruited for muscle …
activity state. This state can conserve energy when the myosin is not recruited for muscle …
[HTML][HTML] Muscle mechanics and thick filament activation: an emerging two-way interaction for the vertebrate striated muscle fine regulation
L Marcucci - International Journal of Molecular Sciences, 2023 - mdpi.com
Contraction in striated muscle is classically described as regulated by calcium-mediated
structural changes in the actin-containing thin filaments, which release the binding sites for …
structural changes in the actin-containing thin filaments, which release the binding sites for …