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 …
The pathogenesis and therapy of muscular dystrophies
Current molecular genomic approaches to human genetic disorders have led to an
explosion in the identification of the genes and their encoded proteins responsible for these …
explosion in the identification of the genes and their encoded proteins responsible for these …
Therapy for Duchenne muscular dystrophy: renewed optimism from genetic approaches
RJ Fairclough, MJ Wood, KE Davies - Nature Reviews Genetics, 2013 - nature.com
Duchenne muscular dystrophy (DMD) is a devastating progressive disease for which there
is currently no effective treatment except palliative therapy. There are several promising …
is currently no effective treatment except palliative therapy. There are several promising …
[HTML][HTML] Pharmacological advances for treatment in Duchenne muscular dystrophy
Highlights•Numerous pathophysiological features of DMD provide different therapeutic
avenues.•Pharmacolougical therapies target all muscles and are applicable to all DMD …
avenues.•Pharmacolougical therapies target all muscles and are applicable to all DMD …
Biglycan: a multivalent proteoglycan providing structure and signals
MV Nastase, MF Young… - Journal of Histochemistry …, 2012 - journals.sagepub.com
Research over the past few years has provided fascinating results indicating that biglycan,
besides being a ubiquitous structural component of the extracellular matrix (ECM), may act …
besides being a ubiquitous structural component of the extracellular matrix (ECM), may act …
[HTML][HTML] Muscle and cardiac therapeutic strategies for Duchenne muscular dystrophy: past, present, and future
A Łoboda, J Dulak - Pharmacological Reports, 2020 - Springer
Background Duchenne muscular dystrophy (DMD) is a severe X-linked neuromuscular
childhood disorder that causes progressive muscle weakness and degeneration and results …
childhood disorder that causes progressive muscle weakness and degeneration and results …
Extracellular matrix regulation in the muscle satellite cell niche
Increasing evidence points to extracellular matrix (ECM) components playing integral roles
in regulating the muscle satellite cell (SC) niche. Even small alterations to the niche ECM …
in regulating the muscle satellite cell (SC) niche. Even small alterations to the niche ECM …
Current and emerging treatment strategies for Duchenne muscular dystrophy
JK Mah - Neuropsychiatric disease and treatment, 2016 - Taylor & Francis
Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in
childhood. It is caused by mutations of the DMD gene, leading to progressive muscle …
childhood. It is caused by mutations of the DMD gene, leading to progressive muscle …
Dystrophinopathies
Dystrophinopathies result from mutations of the DMD gene that primarily affect skeletal
muscle but also affect heart, brain, and smooth muscle. Advances in the genetic analysis of …
muscle but also affect heart, brain, and smooth muscle. Advances in the genetic analysis of …
[HTML][HTML] Daily treatment with SMTC1100, a novel small molecule utrophin upregulator, dramatically reduces the dystrophic symptoms in the mdx mouse
JM Tinsley, RJ Fairclough, R Storer, FJ Wilkes… - PloS one, 2011 - journals.plos.org
Background Duchenne muscular dystrophy (DMD) is a lethal, progressive muscle wasting
disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the …
disease caused by a loss of sarcolemmal bound dystrophin, which results in the death of the …