Membrane glucocorticoid receptors are localised in the extracellular matrix and signal through the MAPK pathway in mammalian skeletal muscle fibres [PDF]
A number of studies have previously proposed the existence of glucocorticoid receptors on the plasma membrane of many cell types including skeletal muscle fibres.
Dietmar Steverding +8 more
core +1 more source
This study presents a novel approach for treating Duchenne muscular dystrophy using mRNA encoding PGC‐1α4. Nanomicelle‐delivered Pgc‐1α4 mRNA enhances muscle damage resistance and mitochondrial activity in dystrophic muscles. This study demonstrates the potential of mRNA therapy for neuromuscular diseases like Duchenne muscular dystrophy and highlights
Xuan Du +7 more
wiley +1 more source
Tadalafil Treatment Delays the Onset of Cardiomyopathy in Dystrophin‐Deficient Hearts
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
Milestone Review: Unlocking the Proteomics of Glycine Receptor Complexes
Glycine receptors (GlyRs) play key roles in brain development, learning/memory, inflammatory pain sensitization, and rhythmic breathing. GlyRs depend upon stable and transient protein–protein interactions that influence synaptic localization, homeostasis, signaling pathways, and receptor function.
Sean D. Fraser +4 more
wiley +1 more source
High Throughput Screening in Duchenne Muscular Dystrophy: From Drug Discovery to Functional Genomics
Centers for the screening of biologically active compounds and genomic libraries are becoming common in the academic setting and have enabled researchers devoted to developing strategies for the treatment of diseases or interested in studying a ...
Thomas J.J. Gintjee +2 more
doaj +1 more source
Post-natal induction of PGC-1α protects against severe muscle dystrophy independently of utrophin [PDF]
Background: Duchenne muscle dystrophy (DMD) afflicts 1 million boys in the US and has few effective treatments. Constitutive transgenic expression of the transcriptional coactivator peroxisome proliferator-activated receptor gamma coactivator (PGC)-1α ...
Arany, Zolt +5 more
core +1 more source
Abstract Absence of the structural protein, dystrophin, results in the neuromuscular disorder Duchenne Muscular Dystrophy (DMD). In addition to progressive skeletal muscle dysfunction, this multisystemic disorder can also result in cognitive deficits and behavioural changes that are likely to be consequences of dystrophin loss from central neurons and ...
Kimberley A. Stephenson +3 more
wiley +1 more source
Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism. [PDF]
Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1 is found ubiquitously.
Blondelle, Jordan +13 more
core +1 more source
Increased levels of interleukin-6 exacerbate the dystrophic phenotype in mdx mice [PDF]
Duchenne muscular dystrophy (DMD) is characterized by progressive lethal muscle degeneration and chronic inflammatory response. The mdx mouse strain has served as the animal model for human DMD. However, while DMD patients undergo extensive necrosis, the
Berardinelli, Maria Grazia +10 more
core +2 more sources
New Horizons in Myotonic Dystrophy Type 1: Cellular Senescence as a Therapeutic Target
Cellular senescence accumulates in the skeletal muscle of patients with myotonic dystrophy Type 1, leading to the production of senescence‐associated secretory phenotype (SASP) factors that have detrimental effects. Targeting these senescent cells with senotherapeutics could restore muscle homeostasis and slow disease progression.
Cécilia Légaré +3 more
wiley +1 more source

