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 …

Use of BODIPY (493/503) to visualize intramuscular lipid droplets in skeletal muscle

EE Spangenburg, SJP Pratt… - BioMed Research …, 2011 - Wiley Online Library
Triglyceride storage is altered across various chronic health conditions necessitating various
techniques to visualize and quantify lipid droplets (LDs). Here, we describe the utilization of …

Pharmacological inhibition of HDAC6 improves muscle phenotypes in dystrophin-deficient mice by downregulating TGF-β via Smad3 acetylation

A Osseni, A Ravel-Chapuis, E Belotti, I Scionti… - Nature …, 2022 - nature.com
The absence of dystrophin in Duchenne muscular dystrophy disrupts the dystrophin-
associated glycoprotein complex resulting in skeletal muscle fiber fragility and atrophy …

TGFβ signaling curbs cell fusion and muscle regeneration

F Girardi, A Taleb, M Ebrahimi, A Datye… - Nature …, 2021 - nature.com
Muscle cell fusion is a multistep process involving cell migration, adhesion, membrane
remodeling and actin-nucleation pathways to generate multinucleated myotubes. However …

Dysferlin stabilizes stress-induced Ca2+ signaling in the transverse tubule membrane

JP Kerr, AP Ziman, AL Mueller… - Proceedings of the …, 2013 - National Acad Sciences
Dysferlinopathies, most commonly limb girdle muscular dystrophy 2B and Miyoshi
myopathy, are degenerative myopathies caused by mutations in the DYSF gene encoding …

Three-dimensional imaging studies in mice identify cellular dynamics of skeletal muscle regeneration

BC Collins, JB Shapiro, MM Scheib, RV Musci… - Developmental Cell, 2024 - cell.com
The function of many organs, including skeletal muscle, depends on their three-dimensional
structure. Muscle regeneration therefore requires not only reestablishment of myofibers but …

Effects of in vivo injury on the neuromuscular junction in healthy and dystrophic muscles

SJP Pratt, SB Shah, CW Ward, MP Inacio… - The Journal of …, 2013 - Wiley Online Library
Key points• Strength loss induced by lengthening contractions is typically attributed to
damaged force‐bearing structures within skeletal muscle. Muscle lacking the structural …

The dystrophin‐glycoprotein complex in the prevention of muscle damage

JD Gumerson, DE Michele - BioMed Research International, 2011 - Wiley Online Library
Muscular dystrophies are genetically diverse but share common phenotypic features of
muscle weakness, degeneration, and progressive decline in muscle function. Previous work …

Pre-and postsynaptic changes in the neuromuscular junction in dystrophic mice

SJP Pratt, AP Valencia, GK Le, SB Shah… - Frontiers in …, 2015 - frontiersin.org
Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disease in which
weakness, increased susceptibility to muscle injury, and inadequate repair appear to …

Mitochondrial oxidative stress impairs contractile function but paradoxically increases muscle mass via fibre branching

B Ahn, R Ranjit, P Premkumar… - Journal of cachexia …, 2019 - Wiley Online Library
Background Excess reactive oxygen species (ROS) and muscle weakness occur in parallel
in multiple pathological conditions. However, the causative role of skeletal muscle …