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Sci. Signal., 15 December 2009
Vol. 2, Issue 101, p. ec402
[DOI: 10.1126/scisignal.2101ec402]


Neurodegeneration An Innervative Small RNA

Paula A. Kiberstis

Science, AAAS, Washington, DC 20005, USA

Amyotrophic lateral sclerosis (ALS) is a relentless disease characterized by progressive degeneration of motor neurons that control muscle movement, leading to muscle atrophy and paralysis. Williams et al. (see the Perspective by Brown) show that a small noncoding RNA that is selectively expressed in skeletal muscle, miR-206, senses motor neuron injury or loss and helps ameliorate resultant muscle damage by promoting regeneration of neuromuscular synapses. Expression of miR-206 was dramatically induced in a mouse model of ALS, and when this RNA was removed from mice by genetic manipulation, the disease progressed at a faster rate. The salutary effects of miR-206 appear to be mediated through a signaling pathway in muscle cells involving histone deacetylase 4 and a fibroblast growth factor modulator, activation of which leads to release of factors that promote nerve-muscle interactions.

A. H. Williams, G. Valdez, V. Moresi, X. Qi, J. McAnally, J. L. Elliott, R. Bassel-Duby, J. R. Sanes, E. N. Olson, MicroRNA-206 delays ALS progression and promotes regeneration of neuromuscular synapses in mice. Science 326, 1549–1554 (2009). [Abstract] [Full Text]

R. H. Brown, A reinnervating microRNA. Science 326, 1494–1495 (2009). [Summary] [Full Text]

Citation: P. A. Kiberstis, An Innervative Small RNA. Sci. Signal. 2, ec402 (2009).

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