http://www.cnr.it/ontology/cnr/individuo/prodotto/ID4172
Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy (Articolo in rivista)
- Type
- Label
- Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy (Articolo in rivista) (literal)
- Anno
- 2007-01-01T00:00:00+01:00 (literal)
- Alternative label
Amy M. Avila, Barrington G. Burnett, Addis A. Taye, Francesca Gabanella, Melanie A. Knight, Parvana Hartenstein, Ziga Cizman, Nicholas A. Di Prospero, Livio Pellizzoni, Kenneth H. Fischbeck, and Charlotte J. Sumner (2007)
Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
(literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Amy M. Avila, Barrington G. Burnett, Addis A. Taye, Francesca Gabanella, Melanie A. Knight, Parvana Hartenstein, Ziga Cizman, Nicholas A. Di Prospero, Livio Pellizzoni, Kenneth H. Fischbeck, and Charlotte J. Sumner (literal)
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- Pubblicazione su rivista internazionale (literal)
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- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Titolo
- Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy (literal)
- Abstract
- The inherited motor neuron disease spinal muscular atrophy (SMA) is caused by mutation of the telomeric
survival motor neuron 1 (SMN1) gene with retention of the centromeric SMN2 gene. We sought to establish
whether the potent and specific hydroxamic acid class of histone deacetylase (HDAC) inhibitors activates
SMN2 gene expression in vivo and modulates the SMA disease phenotype when delivered after disease onset.
Single intraperitoneal doses of 10 mg/kg trichostatin A (TSA) in nontransgenic and SMA model mice resulted
in increased levels of acetylated H3 and H4 histones and modest increases in SMN gene expression. Repeated
daily doses of TSA caused increases in both SMN2-derived transcript and SMN protein levels in neural tissues
and muscle, which were associated with an improvement in small nuclear ribonucleoprotein (snRNP)
assembly. When TSA was delivered daily beginning on P5, after the onset of weight loss and motor deficit,
there was improved survival, attenuated weight loss, and enhanced motor behavior. Pathological analysis
showed increased myofiber size and number and increased anterior horn cell size. These results indicate that
the hydroxamic acid class of HDAC inhibitors activates SMN2 gene expression in vivo and has an ameliorating
effect on the SMA disease phenotype when administered after disease onset. (literal)
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