[HTML][HTML] Role of TGF-β signaling in inherited and acquired myopathies

TN Burks, RD Cohn - Skeletal muscle, 2011 - Springer
… , and myostatin is linked to various features of muscle pathology, … will elaborate on findings
in Duchenne and limb-girdle MDs. … breeding myostatin-null mice to several mouse models of …

AAV-mediated delivery of a mutated myostatin propeptide ameliorates calpain 3 but not α-sarcoglycan deficiency

M Bartoli, J Poupiot, A Vulin, F Fougerousse, L Arandel… - Gene therapy, 2007 - nature.com
… in animal models of two limb-girdle muscular dystrophies: … growth, we assessed its
beneficial effect on pathology in α-… in a mouse model of limb-girdle muscular dystrophy type 2A. …

Anti-latent TGFβ binding protein 4 antibody improves muscle function and reduces muscle fibrosis in muscular dystrophy

AR Demonbreun, KS Fallon, CC Oosterbaan… - Science translational …, 2021 - science.org
… We previously developed a mouse model of DMD thatthat the combination of targeting
TGFβs and myostatin due to … and sarcoglycan-mediated limb girdle muscular dystrophy (LGMD) …

A phase I/IItrial of MYO‐029 in adult subjects with muscular dystrophy

KR Wagner, JL Fleckenstein, AA Amato… - Annals of …, 2008 - Wiley Online Library
… inhibitor of myostatin, designed to increase muscle mass and … In the mdx mouse model
of muscular dystrophy, animals … (FSHD), and limb-girdle muscular dystrophy (LGMD). A …

Prospect of gene therapy for cardiomyopathy in hereditary muscular dystrophy

Y Yue, IM Binalsheikh, SB Leach… - Expert opinion on …, 2016 - Taylor & Francis
limb-girdle and congenital muscular dystrophies, and myotonic … α-dystroglycan glycosylation
in the mouse model and human cells.[… that myostatin blockade benefits a dystrophic heart. …

Dysferlin and animal models for dysferlinopathy

K Kobayashi, T Izawa, M Kuwamura… - … of toxicologic pathology, 2012 - jstage.jst.go.jp
… The murine models are useful in verification experiments for … the histopathological lesions
are localized in the limb-girdle … to myostatin, MYO-029, has been conducted in adult muscular

Prospect for pharmacological therapies to treat skeletal muscle dysfunction

MN Meriggioli, R Roubenoff - Calcified tissue international, 2015 - Springer
Limb-girdle muscular dystrophies (LGMDs) are a group of … In two mouse models of
sarcoglycanopathy, post-natal inhibition of myostatin increased muscle mass [29, 30] and improved

Overexpression of Galgt2 reduces dystrophic pathology in the skeletal muscles of alpha sarcoglycan-deficient mice

R Xu, S DeVries, M Camboni, PT Martin - … American journal of pathology, 2009 - Elsevier
… ), a mouse model for limb girdle muscular dystrophy 2D. Galgt2 … a similar weight of muscle
with SDS/urea buffer (2% SDS, 4 … mdx mice but not in dy/dy mice,18, 86 myostatin inhibition is …

Wasting mechanisms in muscular dystrophy

J Shin, MM Tajrishi, Y Ogura, A Kumar - The international journal of …, 2013 - Elsevier
… −/− model of limb-girdle muscular dystrophy 2F (LGMD2F). … The role of myostatin in
pathogenesis of muscular dystrophy … be beneficial in mouse models, it did not restore full function. …

The myostatin gene: physiology and pharmacological relevance

D Joulia-Ekaza, G Cabello - Current opinion in pharmacology, 2007 - Elsevier
… the activin receptor ActRIIB and Smads 2 and 3. Approaches … -deficient mouse model of
limb-girdle muscular dystrophy 1C… model, does not improve all aspects of muscle pathology; this …