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- Title
Nuclear positioning in muscle development and disease.
- Authors
Folker, Eric S.; Baylies, Mary K.
- Abstract
Muscle disease asa group is characterized bymuscle weakness, muscle loss, and impaired muscle function. Although the phenotype is the same, the under lying cellular pathologies, and the molecular causes of these pathologies, are diverse. One common feature of many muscle disorders is the mispositioning of myonuclei. In unaffected individuals, myonuclei are spaced throughout the periphery of the muscle fiber such that the distance between nuclei is maximized. However, in diseased muscles, the nuclei are often clustered with in the center of the muscle cell. Although this phenotype has been acknowledged for several decades, it is often ignored as a contributor to muscle weakness. Rather, these nuclei are taken only as a sign of muscle repair. Here we review the evidence that mispositioned myonuclei are not merely a symptom of muscle disease but also a cause. Additionally, we review the working models for how myonuclei move from two different perspectives: from that of the nuclei and from that of the cytoskeleton. We further compare and contrast these mechanisms with the mechanisms of nuclear movement in other cell types both to draw general themes for nuclear moement and to identify muscle-specific considerations. Finally, we focus on factors that can be linked to muscle disease and find that genes that regulate myonuclear movement and positioning have been linked to muscuar dystrophy. Although the cause-effect relationship is largely speculative, recent data indicate that the position of nuclei should no longer be considered only a means to diagnose muscle disease.
- Subjects
MUSCLE diseases; MUSCLE cells; NUCLEAR matrix; CELL nuclei; CELL physiology
- Publication
Frontiers in Physiology, 2013, Vol 4, p1
- ISSN
1664-042X
- Publication type
Article
- DOI
10.3389/fphys.2013.00363