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Article

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

Human molecular genetics - 15 Sept 2011

Kwon Deborah Y, Motley William W, Fischbeck Kenneth H, Burnett Barrington G

Abstract excerpt

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 motor function, which was associated with reduced spinal cord and muscle pathology and improved neuromuscular...

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Identifiers

PMID: 21693563
DOI: 10.1093/hmg/ddr288
PMCID: PMC3159550

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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