Understanding the Process of Fibrosis in Duchenne Muscular Dystrophy
Joint Authors
Guerra, Joana
Serrano, Antonio L.
Pessina, Patrizia
Kharraz, Yacine
Muñoz-Cánoves, Pura
Source
Issue
Vol. 2014, Issue 2014 (31 Dec. 2014), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2014-05-03
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
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 with chronic pathologies and occur in a large variety of vital organs and tissues, including skeletal muscle.
In human muscle, fibrosis is most readily associated with the severe muscle wasting disorder Duchenne muscular dystrophy (DMD), caused by loss of dystrophin gene function.
In DMD, skeletal muscle degenerates and is infiltrated by inflammatory cells and the functions of the muscle stem cells (satellite cells) become impeded and fibrogenic cells hyperproliferate and are overactivated, leading to the substitution of skeletal muscle with nonfunctional fibrotic tissue.
Here, we review new developments in our understanding of the mechanisms leading to fibrosis in DMD and several recent advances towards reverting it, as potential treatments to attenuate disease progression.
American Psychological Association (APA)
Kharraz, Yacine& Guerra, Joana& Pessina, Patrizia& Serrano, Antonio L.& Muñoz-Cánoves, Pura. 2014. Understanding the Process of Fibrosis in Duchenne Muscular Dystrophy. BioMed Research International،Vol. 2014, no. 2014, pp.1-11.
https://search.emarefa.net/detail/BIM-512059
Modern Language Association (MLA)
Kharraz, Yacine…[et al.]. Understanding the Process of Fibrosis in Duchenne Muscular Dystrophy. BioMed Research International No. 2014 (2014), pp.1-11.
https://search.emarefa.net/detail/BIM-512059
American Medical Association (AMA)
Kharraz, Yacine& Guerra, Joana& Pessina, Patrizia& Serrano, Antonio L.& Muñoz-Cánoves, Pura. Understanding the Process of Fibrosis in Duchenne Muscular Dystrophy. BioMed Research International. 2014. Vol. 2014, no. 2014, pp.1-11.
https://search.emarefa.net/detail/BIM-512059
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-512059