Myostatin blockade improves function but not histopathology in a murine model of limbgirdle muscular dystrophy 2C

S Bogdanovich, EM McNally… - Muscle & Nerve: Official …, 2008 - Wiley Online Library
… strategies such as magnetic resonance imaging that allow monitoring of fibrosis in vivo, …
.2, 36 In this study we tested antibody-mediated myostatin blockade on Sgcg −/− mice, a model

Age-dependent effect of myostatin blockade on disease severity in a murine model of limb-girdle muscular dystrophy

SA Parsons, DP Millay, MA Sargent, EM McNally… - … journal of pathology, 2006 - Elsevier
… seen from analysis of three to four mice per genotype per marker, although data from only
two mice in each group are shown (mouse 1 and mouse 2). L7 is a housekeeping control …

[HTML][HTML] Natural disease history of mouse models for limb girdle muscular dystrophy types 2D and 2F

S Pasteuning-Vuhman, K Putker… - PloS one, 2017 - journals.plos.org
mouse was anesthetized with 2% isoflurane and function of … muscle pathology in LGMD
mouse models. In contrast to TGF-β, … test regime, since myostatin is profoundly involved in whole …

The therapeutic potential of soluble activin type IIB receptor treatment in a limb girdle muscular dystrophy type 2D mouse model

A Alqallaf, S Engelbeen, A Palo, F Cutrupi… - Neuromuscular …, 2022 - Elsevier
… We hypothesized that the attenuation of myostatin would … there was no improvement of
muscle function or histopathology2) was diluted in blocking wash buffer and incubated in the …

[PDF][PDF] Towards therapies for muscular dystrophies: targeting TGF-beta and myostatin signalling to improve muscle quality and development of reliable outcome …

S Pasteuning-Vuhman - 2018 - scholarlypublications …
… In milder types of limb girdle muscular dystrophies with later … Secondary pathology of muscular
dystrophy and therapeutic … The DBA/2-mdx model is currently being further characterized …

[HTML][HTML] Myostatin inhibition by a follistatin-derived peptide ameliorates the pathophysiology of muscular dystrophy model mice

K Tsuchida - Acta Myologica, 2008 - ncbi.nlm.nih.gov
… mice, limb-girdle muscular dystrophy (LGMD) 1C model mice … , elimination of myostatin did
not recover the pathology in … Only 2 weeks are required for the soluble form of ACVR2B to …

[HTML][HTML] Intermittent glucocorticoid dosing improves muscle repair and function in mice with limb-girdle muscular dystrophy

M Quattrocelli, IM Salamone, PG Page… - … Journal of Pathology, 2017 - Elsevier
… , and the regenerative capacity of primary muscle cells. We found that in murine models of
LGMD 2B and 2C, daily prednisone dosing reduced muscle damage and fibroinflammatory …

[HTML][HTML] The limbgirdle muscular dystrophies: is treatment on the horizon?

ML Chu, E Moran - Neurotherapeutics, 2018 - Elsevier
… have historically been classified into 2 main categories based on the … In this study, they
showed that myostatin inhibition by … of muscle pathology in mouse models of Duchenne muscular

Long-term enhancement of skeletal muscle mass and strength by single gene administration of myostatin inhibitors

AM Haidet, L Rizo, C Handy… - Proceedings of the …, 2008 - National Acad Sciences
… in normal and dystrophic mouse models for >2 years, even … mdx animals reversed muscle
pathology and improved strength, … in the mdx mouse model of Duchenne muscular dystrophy (…

[HTML][HTML] DNA-mediated gene therapy in a mouse model of limb girdle muscular dystrophy 2B

J Ma, C Pichavant, H du Bois, M Bhakta… - … therapy Methods & …, 2017 - cell.com
muscle known as limb girdle muscular dystrophy 2B. To achieve gene delivery of plasmids
encoding dysferlin to hind limb muscles of … Figure 2C indicates that the average luciferase …