Sarcomere dysfunction in nemaline myopathy

JM de Winter, CAC Ottenheijm - Journal of Neuromuscular …, 2017 - content.iospress.com
Nemaline myopathy (NM) is among the most common non-dystrophic congenital
myopathies (incidence 1: 50.000). Hallmark features of NM are skeletal muscle weakness …

Treatments targeting inotropy: a position paper of the Committees on Translational Research and Acute Heart Failure of the Heart Failure Association of the European …

C Maack, T Eschenhagen, N Hamdani… - European heart …, 2019 - academic.oup.com
Acute heart failure (HF) and in particular, cardiogenic shock are associated with high
morbidity and mortality. A therapeutic dilemma is that the use of positive inotropic agents …

[HTML][HTML] Istaroxime for patients with acute heart failure: a systematic review and meta-analysis of randomized controlled trials

M Abuelazm, S Ali, MM AlBarakat, A Mahmoud… - Diseases, 2023 - mdpi.com
Istaroxime, an intravenous inotropic agent with a dual mechanism—increasing both
cardiomyocyte contractility and relaxation—is a novel treatment for acute heart failure (AHF) …

Changes in the dynamics of the cardiac troponin C molecule explain the effects of Ca2+-sensitizing mutations

CM Stevens, K Rayani, G Singh, B Lotfalisalmasi… - Journal of Biological …, 2017 - jbc.org
Cardiac troponin C (cTnC) is the regulatory protein that initiates cardiac contraction in
response to Ca 2+. TnC binding Ca 2+ initiates a cascade of protein–protein interactions …

Sarcomere integrated biosensor detects myofilament-activating ligands in real time during twitch contractions in live cardiac muscle

AD Vetter, AA Martin, BR Thompson… - Journal of molecular and …, 2020 - Elsevier
The sarcomere is the functional unit of cardiac muscle, essential for normal heart function.
To date, it has not been possible to study, in real time, thin filament-based activation …

Reversible Covalent Binding to Cardiac Troponin C by the Ca2+-Sensitizer Levosimendan

IM Robertson, SE Pineda-Sanabria, Z Yan… - Biochemistry, 2016 - ACS Publications
The binding of Ca2+ to cardiac troponin C (cTnC) triggers contraction in heart muscle. In the
diseased heart, the myocardium is often desensitized to Ca2+, which leads to impaired …

[HTML][HTML] Probing the mechanism of cardiovascular drugs using a covalent levosimendan analog

SE Pineda-Sanabria, IM Robertson, YB Sun… - Journal of molecular and …, 2016 - Elsevier
One approach to improve contraction in the failing heart is the administration of calcium (Ca
2+) sensitizers. Although it is known that levosimendan and other sensitizers bind to …

Short-term efficacy and safety of levosimendan in patients with chronic systolic heart failure

JD Zhang, XR Cui, XH Yang, RB Li, M Jia… - … Journal of Africa, 2020 - journals.co.za
The objective was to investigate and evaluate the short-term efficacy and safety of
levosimendan in patients with chronic systolic heart failure. Forty-nine patients with chronic …

Dynamic Equilibrium of Cardiac Troponin C's Hydrophobic Cleft and Its Modulation by Ca2+ Sensitizers and a Ca2+ Sensitivity Blunting Phosphomimic, cTnT(T204E …

W Schlecht, WJ Dong - Bioconjugate chemistry, 2017 - ACS Publications
Several studies have suggested that conformational dynamics are important in the
regulation of thin filament activation in cardiac troponin C (cTnC); however, little direct …

[КНИГА][B] A fret investigation into molecular mechanisms of cardiac troponin activation in reconstituted thin filaments

ME Moutsoglou - 2016 - search.proquest.com
Cardiomyopathies (CM) are the leading cause of death in America, and can develop from
mutations in sarcomeric proteins, leading to altered protein structure and function. Current …