Differential Membrane Localization and Intermolecular Associations of α-Dystrobrevin Isoforms in Skeletal Muscle [PDF]
, 1998 α-Dystrobrevin is both a dystrophin homologue and a component of the dystrophin protein complex. Alternative splicing yields five forms, of which two predominate in skeletal muscle: full-length α-dystrobrevin-1 (84 kD), and COOH-terminal truncated α ...
Adams +43 more
core +3 more sources
Muscle‐Derived Bioactive Factors: MyoEVs and Myokines
Cell Proliferation, Volume 58, Issue 3, March 2025.Overview of the functions and applications of myokines and MyoEVs.
Xupeng Liu +3 more
wiley +1 more source
Functional characterisation of filamentous actin probe expression in neuronal cells. [PDF]
PLoS ONE, 2017 Genetically encoded filamentous actin probes, Lifeact, Utrophin and F-tractin, are used as tools to label the actin cytoskeleton. Recent evidence in several different cell types indicates that these probes can cause changes in filamentous actin dynamics,
Shrujna Patel +8 more
doaj +1 more source
Molecular heterogeneity of the dystrophin-associated protein complex in the mouse kidney nephron: differential alterations in the absence of utrophin and dystrophin [PDF]
, 2018 The dystrophin-associated protein complex (DPC) consisting of syntrophin, dystrobrevin, and dystroglycan isoforms is associated either with dystrophin or its homolog utrophin.
Fritschy, Jean-Marc +2 more
core
International Journal of Experimental Pathology, Volume 106, Issue 2, March 2025.Abstract Contributions made by the dystrophin‐associated glycoprotein complex (DGC) to cell–cell and cell‐extracellular matrix (ECM) interactions are vital in development, homeostasis and pathobiology. This review explores how DGC functions may extend to skeletal pathophysiology by appraising the known roles of its major ECM ligands, and likely ...
Mark Hopkinson, Andrew A. Pitsillides
wiley +1 more source
Utrophin is lacking at the neuromuscular junctions in the extraocular muscles of normal cat: artefact or true? [PDF]
, 2018 Extraocular muscles (EOM) are typically spared in Duchenne muscular dystrophy. We hypothesized that this might be due to different patterns of utrophin expression.
Assadi, Maziar, Müntener, Markus
core
Cytokines and chemokines as regulators of skeletal muscle inflammation: presenting the case of Duchenne muscular dystrophy [PDF]
, 2013 Duchenne muscular dystrophy is a severe inherited muscle disease that affects 1 in 3500 boys worldwide. Infiltration of skeletal muscle by inflammatory cells is an important facet of disease pathophysiology and is strongly associated with disease ...
De Bleecker, Jan, De Paepe, Boel
core +3 more sources
Gene therapy for genetic diseases: challenges and future directions
MedComm, Volume 6, Issue 2, February 2025.The graphical abstract provides an overview of gene therapy approaches, detailing the components of the therapy and the various delivery routes. Both in vivo and ex vivo strategies facilitate the implementation of gene replacement, gene suppression, gene supplementation, and gene editing.
Beibei Qie +4 more
wiley +1 more source
Tadalafil Treatment Delays the Onset of Cardiomyopathy in Dystrophin‐Deficient Hearts
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2016 BackgroundCardiomyopathy is a leading cause of mortality among Duchenne muscular dystrophy patients and lacks effective therapies. Phosphodiesterase type 5 is implicated in dystrophic pathology, and the phosphodiesterase type 5 inhibitor tadalafil has ...
David W. Hammers +5 more
doaj +1 more source
The Journal of Pathology, Volume 265, Issue 1, Page 1-13, January 2025.Abstract Duchenne muscular dystrophy (DMD) is caused by the absence of the full form of the dystrophin protein, which is essential for maintaining the structural integrity of muscle cells, including those in the heart and respiratory system. Despite progress in understanding the molecular mechanisms associated with DMD, myocardial insufficiency ...
Marika Milan +25 more
wiley +1 more source