Physiology of intracellular calcium buffering

D Eisner, E Neher, H Taschenberger… - Physiological …, 2023 - journals.physiology.org
Calcium signaling underlies much of physiology. Almost all the Ca2+ in the cytoplasm is
bound to buffers, with typically only∼ 1% being freely ionized at resting levels in most cells …

Calcium buffering in the heart in health and disease

GL Smith, DA Eisner - Circulation, 2019 - Am Heart Assoc
Changes of intracellular Ca2+ concentration regulate many aspects of cardiac myocyte
function. About 99% of the cytoplasmic calcium in cardiac myocytes is bound to buffers, and …

Extracellular matrix from hypertrophic myocardium provokes impaired twitch dynamics in healthy cardiomyocytes

LR Sewanan, J Schwan, J Kluger, J Park… - JACC: Basic to …, 2019 - jacc.org
Hypertrophic cardiomyopathy (HCM) is often caused by single sarcomeric gene mutations
that affect muscle contraction. Pharmacological correction of mutation effects prevents but …

Modeling hypertrophic cardiomyopathy with human cardiomyocytes derived from induced pluripotent stem cells

J Li, X Feng, X Wei - Stem Cell Research & Therapy, 2022 - Springer
One of the obstacles in studying the pathogenesis of hypertrophic cardiomyopathy (HCM) is
the poor availability of myocardial tissue samples at the early stages of disease …

Human induced pluripotent stem-cell-derived cardiomyocytes as models for genetic cardiomyopathies

A Brodehl, H Ebbinghaus, MA Deutsch… - International journal of …, 2019 - mdpi.com
In the last few decades, many pathogenic or likely pathogenic genetic mutations in over
hundred different genes have been described for non-ischemic, genetic cardiomyopathies …

Molecular and cellular evidence for the impact of a hypertrophic cardiomyopathy-associated RAF1 variant on the structure and function of contractile machinery in …

S Nakhaei-Rad, F Haghighi, F Bazgir… - Communications …, 2023 - nature.com
Noonan syndrome (NS), the most common among RASopathies, is caused by germline
variants in genes encoding components of the RAS-MAPK pathway. Distinct variants …

Ion Channel Impairment and Myofilament Ca2+ Sensitization: Two Parallel Mechanisms Underlying Arrhythmogenesis in Hypertrophic Cardiomyopathy

L Santini, R Coppini, E Cerbai - Cells, 2021 - mdpi.com
Life-threatening ventricular arrhythmias are the main clinical burden in patients with
hypertrophic cardiomyopathy (HCM), and frequently occur in young patients with mild …

Omecamtiv mecarbil evokes diastolic dysfunction and leads to periodic electromechanical alternans

GÁ Fülöp, A Oláh, T Csipo, Á Kovács… - Basic research in …, 2021 - Springer
Omecamtiv mecarbil (OM) is a promising novel drug for improving cardiac contractility. We
tested the therapeutic range of OM and identified previously unrecognized side effects. The …

A perspective on Notch signalling in progression and arrhythmogenesis in familial hypertrophic and dilated cardiomyopathies

P Langa, S Shafaattalab… - … of the Royal …, 2023 - royalsocietypublishing.org
In this perspective, we discussed emerging data indicating a role for Notch signalling in
inherited disorders of the heart failure with focus on hypertrophic cardiomyopathy (HCM) …

Modelling sarcomeric cardiomyopathies with human cardiomyocytes derived from induced pluripotent stem cells

LR Sewanan, SG Campbell - The Journal of physiology, 2020 - Wiley Online Library
Cardiomyocytes derived from human induced pluripotent stem cells (iPSCs) provide a
unique opportunity to understand the pathophysiological effects of genetic cardiomyopathy …