Skeletal muscle in health and disease
J Morgan, T Partridge - Disease models & mechanisms, 2020 - journals.biologists.com
Skeletal muscle fibres are multinucleated cells that contain postmitotic nuclei (ie they are no
longer able to divide) and perform muscle contraction. They are formed by fusion of muscle …
longer able to divide) and perform muscle contraction. They are formed by fusion of muscle …
The mdx mouse model as a surrogate for D uchenne muscular dystrophy
TA Partridge - The FEBS journal, 2013 - Wiley Online Library
Research into fundamental principles and the testing of therapeutic hypotheses for treatment
of human disease is commonly performed on mouse models of human diseases. Although …
of human disease is commonly performed on mouse models of human diseases. Although …
VBP15, a novel anti‐inflammatory and membrane‐stabilizer, improves muscular dystrophy without side effects
CR Heier, JM Damsker, Q Yu… - EMBO molecular …, 2013 - embopress.org
Absence of dystrophin makes skeletal muscle more susceptible to injury, resulting in
breaches of the plasma membrane and chronic inflammation in Duchenne muscular …
breaches of the plasma membrane and chronic inflammation in Duchenne muscular …
Oxidative stress and pathology in muscular dystrophies: focus on protein thiol oxidation and dysferlinopathies
JR Terrill, HG Radley‐Crabb, T Iwasaki… - The FEBS …, 2013 - Wiley Online Library
The muscular dystrophies comprise more than 30 clinical disorders that are characterized by
progressive skeletal muscle wasting and degeneration. Although the genetic basis for many …
progressive skeletal muscle wasting and degeneration. Although the genetic basis for many …
Dystrophin-deficient pigs provide new insights into the hierarchy of physiological derangements of dystrophic muscle
N Klymiuk, A Blutke, A Graf, S Krause… - Human molecular …, 2013 - academic.oup.com
Duchenne muscular dystrophy (DMD) is caused by mutations in the X-linked dystrophin
(DMD) gene. The absence of dystrophin protein leads to progressive muscle weakness and …
(DMD) gene. The absence of dystrophin protein leads to progressive muscle weakness and …
Ferlins: regulators of vesicle fusion for auditory neurotransmission, receptor trafficking and membrane repair
Ferlins are a family of multiple C 2 domain proteins with emerging roles in vesicle fusion and
membrane trafficking. Ferlin mutations are associated with muscular dystrophy (dysferlin) …
membrane trafficking. Ferlin mutations are associated with muscular dystrophy (dysferlin) …
Slow or fast: Implications of myofibre type and associated differences for manifestation of neuromuscular disorders
Many neuromuscular disorders can have a differential impact on a specific myofibre type,
forming the central premise of this review. The many different skeletal muscles in mammals …
forming the central premise of this review. The many different skeletal muscles in mammals …
[HTML][HTML] Novel and optimized strategies for inducing fibrosis in vivo: focus on Duchenne Muscular Dystrophy
Background Fibrosis, an excessive collagen accumulation, results in scar formation,
impairing function of vital organs and tissues. Fibrosis is a hallmark of muscular dystrophies …
impairing function of vital organs and tissues. Fibrosis is a hallmark of muscular dystrophies …
Anti-inflammatory drugs for Duchenne muscular dystrophy: focus on skeletal muscle-releasing factors
S Miyatake, Y Shimizu-Motohashi… - Drug design …, 2016 - Taylor & Francis
Duchenne muscular dystrophy (DMD), an incurable and a progressive muscle wasting
disease, is caused by the absence of dystrophin protein, leading to recurrent muscle fiber …
disease, is caused by the absence of dystrophin protein, leading to recurrent muscle fiber …
[HTML][HTML] Lipid accumulation in dysferlin-deficient muscles
MD Grounds, JR Terrill, HG Radley-Crabb… - The American Journal of …, 2014 - Elsevier
Dysferlin is a membrane associated protein involved in vesicle trafficking and fusion. Defects
in dysferlin result in limb-girdle muscular dystrophy type 2B and Miyoshi myopathy in …
in dysferlin result in limb-girdle muscular dystrophy type 2B and Miyoshi myopathy in …