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 …

The sliding filament theory since Andrew Huxley: multiscale and multidisciplinary muscle research

JD Powers, SA Malingen, M Regnier… - Annual review of …, 2021 - annualreviews.org
Two groundbreaking papers published in 1954 laid out the theory of the mechanism of
muscle contraction based on force-generating interactions between myofilaments in the …

Deciphering the super relaxed state of human β-cardiac myosin and the mode of action of mavacamten from myosin molecules to muscle fibers

RL Anderson, DV Trivedi, SS Sarkar… - Proceedings of the …, 2018 - National Acad Sciences
Mutations in β-cardiac myosin, the predominant motor protein for human heart contraction,
can alter power output and cause cardiomyopathy. However, measurements of the intrinsic …

[HTML][HTML] Effects of MYBPC3 loss-of-function mutations preceding hypertrophic cardiomyopathy

AS Helms, VT Tang, TS O'Leary, S Friedline… - JCI insight, 2020 - ncbi.nlm.nih.gov
Mutations in cardiac myosin binding protein C (MyBP-C, encoded by MYBPC3) are the most
common cause of hypertrophic cardiomyopathy (HCM). Most MYBPC3 mutations result in …

RPL3L-containing ribosomes determine translation elongation dynamics required for cardiac function

C Shiraishi, A Matsumoto, K Ichihara… - Nature …, 2023 - nature.com
Although several ribosomal protein paralogs are expressed in a tissue-specific manner, how
these proteins affect translation and why they are required only in certain tissues have …

MyBP-C: one protein to govern them all

L Heling, MA Geeves, NM Kad - Journal of muscle research and cell …, 2020 - Springer
The heart is an extraordinarily versatile pump, finely tuned to respond to a multitude of
demands. Given the heart pumps without rest for decades its efficiency is particularly …

Contractility and calcium transient maturation in the human iPSC-derived cardiac microfibers

D Strimaityte, C Tu, A Yanez, I Itzhaki… - … applied materials & …, 2022 - ACS Publications
Human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) are considered
immature in the sarcomere organization, contractile machinery, calcium transient, and …

Phosphorylation and calcium antagonistically tune myosin-binding protein C's structure and function

MJ Previs, JY Mun, AJ Michalek… - Proceedings of the …, 2016 - National Acad Sciences
During each heartbeat, cardiac contractility results from calcium-activated sliding of actin thin
filaments toward the centers of myosin thick filaments to shorten cellular length. Cardiac …

Spatial and Functional Distribution of MYBPC3 Pathogenic Variants and Clinical Outcomes in Patients With Hypertrophic Cardiomyopathy

AS Helms, AD Thompson, AA Glazier… - Circulation: Genomic …, 2020 - Am Heart Assoc
Background: Pathogenic variants in MYBPC3, encoding cardiac MyBP-C (myosin binding
protein C), are the most common cause of familial hypertrophic cardiomyopathy. A large …

Myosin-binding protein H-like regulates myosin-binding protein distribution and function in atrial cardiomyocytes

DY Barefield, P Tonino, KC Woulfe… - Proceedings of the …, 2023 - National Acad Sciences
Mutations in atrial-enriched genes can cause a primary atrial myopathy that can contribute to
overall cardiovascular dysfunction. MYBPHL encodes myosin-binding protein H-like (MyBP …