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 …

A muscle stem cell for every muscle: variability of satellite cell biology among different muscle groups

ME Randolph, GK Pavlath - Frontiers in aging neuroscience, 2015 - frontiersin.org
The human body contains approximately 640 individual skeletal muscles. Despite the fact
that all of these muscles are composed of striated muscle tissue, the biology of these …

Pharmacological inhibition of HDAC6 improves muscle phenotypes in dystrophin-deficient mice by downregulating TGF-β via Smad3 acetylation

A Osseni, A Ravel-Chapuis, E Belotti, I Scionti… - Nature …, 2022 - nature.com
The absence of dystrophin in Duchenne muscular dystrophy disrupts the dystrophin-
associated glycoprotein complex resulting in skeletal muscle fiber fragility and atrophy …

Mechanisms of larynx and vocal fold development and pathogenesis

V Lungova, SL Thibeault - Cellular and Molecular Life Sciences, 2020 - Springer
The larynx and vocal folds sit at the crossroad between digestive and respiratory tracts and
fulfill multiple functions related to breathing, protection and phonation. They develop at the …

Genetic deletion of muscle RANK or selective inhibition of RANKL is not as effective as full-length OPG-fc in mitigating muscular dystrophy

SS Dufresne, A Boulanger-Piette, S Bossé… - Acta Neuropathologica …, 2018 - Springer
Although there is a strong association between osteoporosis and skeletal muscle
atrophy/dysfunction, the functional relevance of a particular biological pathway that …

Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy

S Carberry, M Zweyer… - International …, 2012 - spandidos-publications.com
Duchenne muscular dystrophy is a lethal genetic disease of childhood caused by primary
abnormalities in the gene coding for the membrane cytoskeletal protein dystrophin. The mdx …

Cardiac and respiratory dysfunction in Duchenne muscular dystrophy and the role of second messengers

M Mosqueira, U Zeiger, M Förderer… - Medicinal research …, 2013 - Wiley Online Library
Duchenne muscular dystrophy (DMD) affects young boys and is characterized by the
absence of dystrophin, a large cytoskeletal protein present in skeletal and cardiac muscle …

Proteomics of the dystrophin-glycoprotein complex and dystrophinopathy

A Holland, S Carberry… - Current Protein and …, 2013 - ingentaconnect.com
The largest human gene is represented by the X-chromosomal dystrophin gene of 2.4
million bases, which encodes for the membrane cytoskeletal protein dystrophin. The …

Chaperoning heat shock proteins: proteomic analysis and relevance for normal and dystrophin‐deficient muscle

H Brinkmeier, K Ohlendieck - PROTEOMICS–Clinical …, 2014 - Wiley Online Library
Molecular chaperones play a key role in normal muscle function and during physiological
adaptations to extensive exercise and numerous forms of cellular stress. The various …

Targeting the muscle-bone unit: filling two needs with one deed in the treatment of Duchenne muscular dystrophy

A Boulanger Piette, D Hamoudi, L Marcadet… - Current osteoporosis …, 2018 - Springer
Abstract Purpose of Review In Duchenne muscular dystrophy (DMD), the progressive
skeletal and cardiac muscle dysfunction and degeneration is accompanied by low bone …