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 …

Troponin structure and function: a view of recent progress

S Marston, JE Zamora - Journal of muscle research and cell motility, 2020 - Springer
The molecular mechanism by which Ca 2+ binding and phosphorylation regulate muscle
contraction through Troponin is not yet fully understood. Revealing the differences between …

Genetic restrictive cardiomyopathy: causes and consequences—an integrative approach

D Cimiotti, H Budde, R Hassoun, K Jaquet - International Journal of …, 2021 - mdpi.com
The sarcomere as the smallest contractile unit is prone to alterations in its functional,
structural and associated proteins. Sarcomeric dysfunction leads to heart failure or …

Thin filament cardiomyopathies: A review of genetics, disease mechanisms, and emerging therapeutics

LK Keyt, JM Duran, QM Bui, C Chen… - Frontiers in …, 2022 - frontiersin.org
All muscle contraction occurs due to the cyclical interaction between sarcomeric thin and
thick filament proteins within the myocyte. The thin filament consists of the proteins actin …

Intersection of the ubiquitin–proteasome system with oxidative stress in cardiovascular disease

M Qiu, J Chen, X Li, J Zhuang - International Journal of Molecular …, 2022 - mdpi.com
Cardiovascular diseases (CVDs) present a major social problem worldwide due to their high
incidence and mortality rate. Many pathophysiological mechanisms are involved in CVDs …

Molecular mechanisms in cardiomyopathy

K Dadson, L Hauck, F Billia - Clinical Science, 2017 - portlandpress.com
Cardiomyopathies represent a heterogeneous group of diseases that negatively affect heart
function. Primary cardiomyopathies specifically target the myocardium, and may arise from …

[HTML][HTML] Restrictive cardiomyopathy: from genetics and clinical overview to animal modeling

M Chintanaphol, BO Orgil, NR Alberson… - Reviews in …, 2022 - imrpress.com
Restrictive cardiomyopathy (RCM), a potentially devastating heart muscle disorder, is
characterized by diastolic dysfunction due to abnormal muscle relaxation and myocardial …

Cardiomyopathic troponin mutations predominantly occur at its interface with actin and tropomyosin

LS Tobacman, A Cammarato - Journal of General Physiology, 2021 - rupress.org
Reversible Ca2+ binding to troponin is the primary on-off switch of the contractile apparatus
of striated muscles, including the heart. Dominant missense mutations in human cardiac …

The Frank–Starling Law: a jigsaw of titin proportions

V Sequeira, J van der Velden - Biophysical reviews, 2017 - Springer
Abstract The Frank–Starling Law dictates that the heart is able to match ejection to the
dynamic changes occurring during cardiac filling, hence efficiently regulating isovolumetric …

Suppression of lusitropy as a disease mechanism in cardiomyopathies

S Marston, JR Pinto - Frontiers in Cardiovascular Medicine, 2023 - frontiersin.org
In cardiac muscle the action of adrenaline on β1 receptors of heart muscle cells is essential
to adjust cardiac output to the body's needs. Adrenergic activation leads to enhanced …