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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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| Main Authors: | , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Oxford University Press
2011
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| Assuntos: | |
| Acesso em linha: | 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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