RhoA/ROCK2 signalling is enhanced by PDGF‐AA in fibro‐adipogenic progenitor cells: implications for Duchenne muscular dystrophy

E Fernández‐Simón, X Suárez‐Calvet… - Journal of Cachexia …, 2022 - Wiley Online Library
Background The lack of dystrophin expression in Duchenne muscular dystrophy (DMD)
induces muscle fibre and replacement by fibro‐adipose tissue. Although the role of some …

RhoA/ROCK signalling activated by ARHGEF3 promotes muscle weakness via autophagy in dystrophic mdx mice

JS You, Y Kim, S Lee, R Bashir… - Journal of Cachexia …, 2023 - Wiley Online Library
Background Duchenne muscular dystrophy (DMD), caused by dystrophin deficiency, leads
to progressive and fatal muscle weakness through yet‐to‐be‐fully deciphered molecular …

[HTML][HTML] Stromal cell-derived factors in Duchenne muscular dystrophy

E Abdel-Salam, IE Abdel-Meguid, R Shatla… - Acta …, 2010 - ncbi.nlm.nih.gov
Duchenne muscular dystrophy (DMD) is characterized by increased muscle damage and an
abnormal blood flow after muscle contraction leading to a state of functional ischemia …

PDGFRα signalling promotes fibrogenic responses in collagen‐producing cells in Duchenne muscular dystrophy

N Ieronimakis, A Hays, A Prasad… - The Journal of …, 2016 - Wiley Online Library
Fibrosis is a characteristic of Duchenne muscular dystrophy (DMD), yet the cellular and
molecular mechanisms responsible for DMD fibrosis are poorly understood. Utilizing the …

Dystrophin restoration in skeletal, heart and skin arrector pili smooth muscle of mdx mice by ZM2 NP–AON complexes

A Ferlini, P Sabatelli, M Fabris, E Bassi, S Falzarano… - Gene Therapy, 2010 - nature.com
Potentially viable therapeutic approaches for Duchenne muscular dystrophy (DMD) are now
within reach. Indeed, clinical trials are currently under way. Two crucial aspects still need to …

Nitric oxide controls fat deposition in dystrophic skeletal muscle by regulating fibro-adipogenic precursor differentiation

N Cordani, V Pisa, L Pozzi, C Sciorati, E Clementi - Stem cells, 2014 - academic.oup.com
Duchenne muscular dystrophy (DMD) is an hereditary disease characterized by loss of
muscle fibers and their progressive substitution by fat and fibrous tissue. Mesenchymal fibro …

Targeting fibrosis in Duchenne muscular dystrophy

L Zhou, H Lu - Journal of Neuropathology & Experimental …, 2010 - academic.oup.com
Duchenne muscular dystrophy (DMD) is the most common genetic muscle disease affecting
1 in 3,500 live male births. It is an X-linked recessive disease caused by a defective …

[HTML][HTML] Total absence of dystrophin expression exacerbates ectopic myofiber calcification and fibrosis and alters macrophage infiltration patterns

CNJ Young, MRF Gosselin, R Rumney… - The American Journal of …, 2020 - Elsevier
Duchenne muscular dystrophy (DMD) causes severe disability and death of young men
because of progressive muscle degeneration aggravated by sterile inflammation. DMD is …

Understanding the process of fibrosis in Duchenne muscular dystrophy

Y Kharraz, J Guerra, P Pessina… - BioMed research …, 2014 - Wiley Online Library
Fibrosis is the aberrant deposition of extracellular matrix (ECM) components during tissue
healing leading to loss of its architecture and function. Fibrotic diseases are often associated …

Insights into the pathogenic secondary symptoms caused by the primary loss of dystrophin

L Forcina, L Pelosi, C Miano, A Musarò - Journal of Functional …, 2017 - mdpi.com
Duchenne muscular dystrophy (DMD) is an X-linked genetic disease in which the dystrophin
gene is mutated, resulting in dysfunctional dystrophin protein. Without dystrophin, the …