Abstract
Mammalian musculature is a very robust and dynamic tissue that goes through many rounds of degeneration and regeneration in an individual’s lifetime. There is a biological program that maintains muscle progenitor cells that, when activated, give rise to intermediate myoblast progeny that consequently differentiate into mature muscle cells. Recent works have provided a picture of the role that microRNAs (miRNAs) play in maintaining aspects of this program. Intriguingly, a subset of these miRNAs is de-regulated in muscular dystrophies (MDs), a group of fatal inherited neuromuscular disorders that are often associated with deficiencies in the Dystrophin (Dys) complex. Apparently, transcriptional expression of many of the muscle specific genes and miRNAs is dependent on chromatin state regulated by the Dys–Syn–nNOS pathway. This puts Dystrophin at the epicenter of a highly regulated program of muscle gene expression in which miRNAs help to coordinate networking between multiple phases of muscle maintenance, degeneration, and regeneration. Therefore, understanding the role of miRNAs in physiology of normal and diseased muscle tissue could be useful for future applications in improving the MD therapies and could open new clinical perspectives.
Highlights
Reviewed by: Francesca Fanini, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Italy Kin Fai Au, Stanford University, USA Hui Ling, muscular dystrophies (MDs) Anderson Cancer Center, USA
A subset of these miRNAs is de-regulated in muscular dystrophies (MDs), a group of fatal inherited neuromuscular disorders that are often associated with deficiencies in the Dystrophin (Dys) complex
FUNCTION OF THE DYSTROPHIN GLYCOPROTEIN COMPLEX Muscular dystrophies (MDs) are a group of fatal inherited diseases (∼30), associated with progressive muscle wasting caused by muscle membrane fragility, abnormal metabolic control, increased oxidative and energetic stress and abnormal proliferation of satellite cells (Campbell, 1995; Cohn and Campbell, 2000; Constantin et al, 2006; Vercherat et al, 2009; Wallace and McNally, 2009)
Summary
Reviewed by: Francesca Fanini, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori, Italy Kin Fai Au, Stanford University, USA Hui Ling, MD Anderson Cancer Center, USA. Another miRNA that is known to regulate muscle terminal differentiation genes is miR-31, which directly targets the 3 -UTR or Dys inactivating the Dys–Syn–nNOS pathway (Cacchiarelli et al, 2011).
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