Mechanisms of muscle degeneration, regeneration, and repair in the muscular dystrophies

GQ Wallace, EM McNally - Annual review of physiology, 2009 - annualreviews.org
To withstand the rigors of contraction, muscle fibers have specialized protein complexes that
buffer against mechanical stress and a multifaceted repair system that is rapidly activated …

Towards developing standard operating procedures for pre-clinical testing in the mdx mouse model of Duchenne muscular dystrophy

MD Grounds, HG Radley, GS Lynch, K Nagaraju… - Neurobiology of …, 2008 - Elsevier
This review discusses various issues to consider when developing standard operating
procedures for pre-clinical studies in the mdx mouse model of Duchenne muscular …

Osteopontin promotes fibrosis in dystrophic mouse muscle by modulating immune cell subsets and intramuscular TGF-β

SA Vetrone, E Montecino-Rodriguez… - The Journal of …, 2009 - Am Soc Clin Investig
Duchenne muscular dystrophy (DMD) is an X-linked, degenerative muscle disease that is
exacerbated by secondary inflammation. Here, we characterized the immunological milieu …

Reduced necrosis of dystrophic muscle by depletion of host neutrophils, or blocking TNFα function with Etanercept in mdx mice

S Hodgetts, H Radley, M Davies, MD Grounds - Neuromuscular Disorders, 2006 - Elsevier
Necrosis of skeletal muscle fibres in the lethal childhood myopathy Duchenne Muscular
Dystrophy results from deficiency of the cell membrane associated protein, dystrophin. We …

Mitochondrial stress responses in Duchenne muscular dystrophy: metabolic dysfunction or adaptive reprogramming?

CA Bellissimo, MC Garibotti… - American Journal of …, 2022 - journals.physiology.org
Mitochondrial stress may be a secondary contributor to muscle weakness in inherited
muscular dystrophies. Duchenne muscular dystrophy has received the majority of attention …

Mesenchymal stem cells as anti-inflammatories: implications for treatment of Duchenne muscular dystrophy

TE Ichim, DT Alexandrescu, F Solano, F Lara… - Cellular …, 2010 - Elsevier
Duchenne muscular dystrophy (DMD) is a lethal X-linked musculodegenerative condition
consisting of an underlying genetic defect whose manifestation is augmented by …

Mammalian animal models for Duchenne muscular dystrophy

R Willmann, S Possekel, J Dubach-Powell… - Neuromuscular …, 2009 - Elsevier
Duchenne muscular dystrophy (DMD) is a fatal neuromuscular disease that affects boys and
leads to early death. In the quest for new treatments that improve the quality of life and in the …

Microdystrophin ameliorates muscular dystrophy in the canine model of duchenne muscular dystrophy

JH Shin, X Pan, CH Hakim, HT Yang, Y Yue, K Zhang… - Molecular therapy, 2013 - cell.com
Dystrophin deficiency results in lethal Duchenne muscular dystrophy (DMD). Substituting
missing dystrophin with abbreviated microdystrophin has dramatically alleviated disease in …

New therapies for Duchenne muscular dystrophy: challenges, prospects and clinical trials

G Cossu, M Sampaolesi - Trends in molecular medicine, 2007 - cell.com
Muscular dystrophies primarily affect skeletal muscle. Mutations in a large number of genes,
mainly encoding cytoskeletal proteins, cause different forms of dystrophy that compromise …

Preclinical drug trials in the mdx mouse: Assessment of reliable and sensitive outcome measures

CF Spurney, H Gordish‐Dressman… - Muscle & Nerve …, 2009 - Wiley Online Library
The availability of animal models for Duchenne muscular dystrophy has led to extensive
preclinical research on potential therapeutics. Few studies have focused on reliability and …