Small angle X-ray diffraction as a tool for structural characterization of muscle disease

W Ma, TC Irving - International Journal of Molecular Sciences, 2022 - mdpi.com
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 …

Nucleus mechanosensing in cardiomyocytes

IL Coscarella, M Landim-Vieira… - International Journal of …, 2023 - mdpi.com
Cardiac muscle contraction is distinct from the contraction of other muscle types. The heart
continuously undergoes contraction–relaxation cycles throughout an animal's lifespan. It …

Myosin-binding protein C regulates the sarcomere lattice and stabilizes the OFF states of myosin heads

AL Hessel, NM Engels, MN Kuehn, D Nissen… - Nature …, 2024 - nature.com
Muscle contraction is produced via the interaction of myofilaments and is regulated so that
muscle performance matches demand. Myosin-binding protein C (MyBP-C) is a long and …

Molecular basis of force-pCa relation in MYL2 cardiomyopathy mice: Role of the super-relaxed state of myosin

CC Yuan, K Kazmierczak, J Liang… - Proceedings of the …, 2022 - National Acad Sciences
In this study, we investigated the role of the super-relaxed (SRX) state of myosin in the
structure–function relationship of sarcomeres in the hearts of mouse models of …

Cardiac troponin T N-domain variant destabilizes the actin interface resulting in disturbed myofilament function

M Landim-Vieira, W Ma, T Song… - Proceedings of the …, 2023 - National Acad Sciences
Missense variant Ile79Asn in human cardiac troponin T (cTnT-I79N) has been associated
with hypertrophic cardiomyopathy and sudden cardiac arrest in juveniles. cTnT-I79N is …

Cardiac troponin activator CK-963 increases cardiac contractility in rats

SE Collibee, A Romero, AR Muci… - Journal of Medicinal …, 2024 - ACS Publications
Novel cardiac troponin activators were identified using a high throughput cardiac myofibril
ATPase assay and confirmed using a series of biochemical and biophysical assays. HTS hit …

Creatine Kinase Equilibration and ΔGATP over an Extended Range of Physiological Conditions: Implications for Cellular Energetics, Signaling, and Muscle …

RW Wiseman, CM Brown, TW Beck, JJ Brault… - International Journal of …, 2023 - mdpi.com
In this report, we establish a straightforward method for estimating the equilibrium constant
for the creatine kinase reaction (CK Keq ″) over wide but physiologically and …

Myofibril orientation as a metric for characterizing heart disease

W Ma, H Gong, V Jani, KH Lee, M Landim-Vieira… - Biophysical journal, 2022 - cell.com
Myocyte disarray is a hallmark of many cardiac disorders. However, the relationship
between alterations in the orientation of individual myofibrils and myofilaments to disease …

Disruption of Z-disc function promotes mechanical dysfunction in human myocardium: evidence for a dual myofilament modulatory role by alpha-actinin 2

M Rodriguez Garcia, J Schmeckpeper… - International Journal of …, 2023 - mdpi.com
The ACTN2 gene encodes α-actinin 2, located in the Z-disc of the sarcomeres in striated
muscle. In this study, we sought to investigate the effects of an ACTN2 missense variant of …

The intrinsically disordered C terminus of troponin T binds to troponin C to modulate myocardial force generation

JR Johnston, M Landim-Vieira, MA Marques… - Journal of Biological …, 2019 - ASBMB
Aberrant regulation of myocardial force production represents an early biomechanical defect
associated with sarcomeric cardiomyopathies, but the molecular mechanisms remain poorly …