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 …

… promotes fast fibre hypertrophy but causes loss of AMP‐activated protein kinase signalling and poor exercise tolerance in a model of limbgirdle muscular dystrophy …

I Kramerova, M Marinov, J Owens… - The Journal of …, 2020 - Wiley Online Library
… - to 2-fold increase that we observed in the same muscles of … to test myostatin inhibition in
the mouse model for LGMD R1. In … histopathology suggesting that mechanisms involved in a …

The failed clinical story of myostatin inhibitors against Duchenne muscular dystrophy: exploring the biology behind the battle

E Rybalka, CA Timpani, DA Debruin, RM Bagaric… - Cells, 2020 - mdpi.com
… manifests as a more severe pathology, which limits functionality… Limb Girdle Muscular
Dystrophy R1 mouse model (C3KO) … However, improved functional testing was not observed in …

Pharmacological Inhibition of Myostatin in a Mouse Model of Typical Nemaline Myopathy Increases Muscle Size and Force

J Lindqvist, H Granzier - International Journal of Molecular Sciences, 2023 - mdpi.com
dystrophy, inclusion body myositis, limb-girdle muscular … exceeded that of untreated wild-type
mice (Figure 2B). We did … -automatic muscle analysis with the segmentation of histology (…

Myostatin/activin receptor ligands in muscle and the development status of attenuating drugs

BD Rodgers, CW Ward - Endocrine Reviews, 2022 - academic.oup.com
… a comorbidity and negative modifier that accelerates pathology in … that suppress muscle
growth, induce muscle atrophy, and … Circulating myostatin levels typically range between 2 and …

MiRNAs, myostatin, and muscle MRI imaging as biomarkers of clinical features in becker muscular dystrophy

R Marozzo, V Pegoraro, C Angelini - Diagnostics, 2020 - mdpi.com
… from BMD is limb-girdle muscular dystrophy, the hallmark of … pathology [28] when there is
consistent damage of muscle … regeneration process in mdx mouse model of DMD following …

Antimyostatin treatment in health and disease: The story of great expectations and limited success

TL Nielsen, J Vissing, TO Krag - Cells, 2021 - mdpi.com
… for myostatin inhibition with respect to the knowledge that the … −/− model of limb-girdle
muscular dystrophy (LGMD) 2C in a … The Sgcd −/− mouse model of LGMD2F also treated with …

[HTML][HTML] The elusive promise of myostatin inhibition for muscular dystrophy

KR Wagner - Current opinion in neurology, 2020 - journals.lww.com
… -deficient mouse model of limb girdle muscular dystrophy (… lacking myostatin did not have
improvement in pathology and … of sufficient treatment duration (up to 2 years) and the lack of …

Single SERCA2a therapy ameliorated dilated cardiomyopathy for 18 months in a mouse model of Duchenne muscular dystrophy

NB Wasala, Y Yue, W Lostal, LP Wasala, N Niranjan… - Molecular therapy, 2020 - cell.com
… was indistinguishable from that of normal mice (Figure 2C). … Skeletal muscle histology was
evaluated by hematoxylin and … in a hamster model of limb girdle muscular dystrophy and to …

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) …