Abstract：
:A number of recent studies have highlighted the importance of autophagy and the ubiquitin-proteasome in the pathogenesis of muscle wasting in different types of inherited muscle disorders. Autophagy is crucial for the removal of dysfunctional organelles and protein aggregates, whereas the ubiquitin-proteasome is important for the quality control of proteins. Post-mitotic tissues, such as skeletal muscle, are particularly susceptible to aged or dysfunctional organelles and aggregation-prone proteins. Therefore, these degradation systems need to be carefully regulated in muscles. Indeed, excessive or defective activity of the autophagy lysosome or ubiquitin-proteasome leads to detrimental effects on muscle homeostasis. A growing number of studies link abnormalities in the regulation of these two pathways to myofiber degeneration and muscle weakness. Understanding the pathogenic role of these degradative systems in each inherited muscle disorder might provide novel therapeutic targets to counteract muscle wasting. In this Commentary, we will discuss the current view on the role of autophagy lysosome and ubiquitin-proteasome in the pathogenesis of myopathies and muscular dystrophies, and how alteration of these degradative systems contribute to muscle wasting in inherited muscle disorders. We will also discuss how modulating autophagy and proteasome might represent a promising strategy for counteracting muscle loss in different diseases.
journal_name
J Cell Scijournal_title
Journal of cell scienceauthors
Sandri M,Coletto L,Grumati P,Bonaldo Pdoi
10.1242/jcs.114041subject
Has Abstractpub_date
2013-12-01 00:00:00pages
5325-33issue
Pt 23eissn
0021-9533issn
1477-9137pii
126/23/5325journal_volume
126pub_type
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journal_title：Journal of cell science
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journal_title：Journal of cell science
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doi：
更新日期：1990-01-01 00:00:00
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更新日期：1998-04-01 00:00:00
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