Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice

Spinal muscular atrophy (SMA) is a neuromuscular disorder caused by reduced levels of the survival motor neuron (SMN) protein. Here we show that the proteasome inhibitor, bortezomib, increases SMN in cultured cells and in peripheral tissues of SMA model mice. Bortezomib-treated animals had improved...

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Hauptverfasser: Kwon, Deborah Y., Motley, William W., Fischbeck, Kenneth H., Burnett, Barrington G.
Format: Artigo
Sprache:Inglês
Veröffentlicht: Oxford University Press 2011
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Articles
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3159550/
https://ncbi.nlm.nih.gov/pubmed/21693563
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddr288
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