Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy
DG Allen, NP Whitehead… - Physiological …, 2016 - journals.physiology.org
Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a
complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further …
complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further …
Metabolic control of cell death
Background For several decades, intermediate metabolism and signal transduction have
been considered two independent entities. On one side stood the catabolic and anabolic …
been considered two independent entities. On one side stood the catabolic and anabolic …
[HTML][HTML] Physiopathology of the permeability transition pore: Molecular mechanisms in human pathology
Mitochondrial permeability transition (MPT) is the sudden loss in the permeability of the
inner mitochondrial membrane (IMM) to low-molecular-weight solutes. Due to osmotic …
inner mitochondrial membrane (IMM) to low-molecular-weight solutes. Due to osmotic …
Early myopathy in Duchenne muscular dystrophy is associated with elevated mitochondrial H2O2 emission during impaired oxidative phosphorylation
MC Hughes, SV Ramos, PC Turnbull… - Journal of cachexia …, 2019 - Wiley Online Library
Background Muscle wasting and weakness in Duchenne muscular dystrophy (DMD) causes
severe locomotor limitations and early death due in part to respiratory muscle failure. Given …
severe locomotor limitations and early death due in part to respiratory muscle failure. Given …
[HTML][HTML] Development, structure, and maintenance of C. elegans body wall muscle
K Gieseler, H Qadota, GM Benian - … the online review of C. elegans …, 2018 - ncbi.nlm.nih.gov
In C. elegans, mutants that are defective in muscle function and/or structure are easy to
detect and analyze since: 1) body wall muscle is essential for locomotion, and 2) muscle …
detect and analyze since: 1) body wall muscle is essential for locomotion, and 2) muscle …
[HTML][HTML] Duchenne muscular dystrophy is associated with the inhibition of calcium uniport in mitochondria and an increased sensitivity of the organelles to the calcium …
MV Dubinin, EY Talanov, KS Tenkov… - … et Biophysica Acta (BBA …, 2020 - Elsevier
Duchenne muscular dystrophy (DMD) is characterized by a pronounced and progressive
degradation of the structure of skeletal muscles, which decreases their strength and lowers …
degradation of the structure of skeletal muscles, which decreases their strength and lowers …
Dysregulation of calcium homeostasis in muscular dystrophies
A Vallejo-Illarramendi, I Toral-Ojeda… - Expert reviews in …, 2014 - cambridge.org
Muscular dystrophies are a group of diseases characterised by the primary wasting of
skeletal muscle, which compromises patient mobility and in the most severe cases originate …
skeletal muscle, which compromises patient mobility and in the most severe cases originate …
Diverse functions of cytochrome c in cell death and disease
Z Zhou, T Arroum, X Luo, R Kang, YJ Lee… - Cell Death & …, 2024 - nature.com
The redox-active protein cytochrome c is a highly positively charged hemoglobin that
regulates cell fate decisions of life and death. Under normal physiological conditions …
regulates cell fate decisions of life and death. Under normal physiological conditions …
[HTML][HTML] Activation of mitochondrial fusion provides a new treatment for mitochondria-related diseases
Mitochondria fragmentation destabilizes mitochondrial membranes, promotes oxidative
stress and facilitates cell death, thereby contributing to the development and the progression …
stress and facilitates cell death, thereby contributing to the development and the progression …
[HTML][HTML] Sulfur amino acid supplementation displays therapeutic potential in a C. elegans model of Duchenne muscular dystrophy
RA Ellwood, L Slade, J Lewis, R Torregrossa… - Communications …, 2022 - nature.com
Mutations in the dystrophin gene cause Duchenne muscular dystrophy (DMD), a common
muscle disease that manifests with muscle weakness, wasting, and degeneration. An …
muscle disease that manifests with muscle weakness, wasting, and degeneration. An …