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Severe SMA mice show organ impairment that cannot be rescued by therapy with the HDACi JNJ-26481585.

Schreml J, Riessland M, Paterno M, Garbes L, Roßbach K, Ackermann B, Krämer J, Somers E, Parson SH, Heller R, Berkessel A, Sterner-Kock A, Wirth B.

Eur J Hum Genet. 2013 Jun;21(6):643-52. doi: 10.1038/ejhg.2012.222. Epub 2012 Oct 17.


Reversible molecular pathology of skeletal muscle in spinal muscular atrophy.

Mutsaers CA, Wishart TM, Lamont DJ, Riessland M, Schreml J, Comley LH, Murray LM, Parson SH, Lochmüller H, Wirth B, Talbot K, Gillingwater TH.

Hum Mol Genet. 2011 Nov 15;20(22):4334-44. doi: 10.1093/hmg/ddr360. Epub 2011 Aug 12.


LBH589 induces up to 10-fold SMN protein levels by several independent mechanisms and is effective even in cells from SMA patients non-responsive to valproate.

Garbes L, Riessland M, Hölker I, Heller R, Hauke J, Tränkle C, Coras R, Blümcke I, Hahnen E, Wirth B.

Hum Mol Genet. 2009 Oct 1;18(19):3645-58. doi: 10.1093/hmg/ddp313. Epub 2009 Jul 7.


Detection of human survival motor neuron (SMN) protein in mice containing the SMN2 transgene: applicability to preclinical therapy development for spinal muscular atrophy.

Mattis VB, Butchbach ME, Lorson CL.

J Neurosci Methods. 2008 Oct 30;175(1):36-43. doi: 10.1016/j.jneumeth.2008.07.024. Epub 2008 Aug 15.


Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy.

Bowerman M, Murray LM, Boyer JG, Anderson CL, Kothary R.

BMC Med. 2012 Mar 7;10:24. doi: 10.1186/1741-7015-10-24.


A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology.

Bowerman M, Murray LM, Beauvais A, Pinheiro B, Kothary R.

Neuromuscul Disord. 2012 Mar;22(3):263-76. doi: 10.1016/j.nmd.2011.09.007. Epub 2011 Nov 8.


Multiple therapeutic effects of valproic acid in spinal muscular atrophy model mice.

Tsai LK, Tsai MS, Ting CH, Li H.

J Mol Med (Berl). 2008 Nov;86(11):1243-54. doi: 10.1007/s00109-008-0388-1. Epub 2008 Jul 23.


Increasing SMN levels using the histone deacetylase inhibitor SAHA ameliorates defects in skeletal muscle microvasculature in a mouse model of severe spinal muscular atrophy.

Somers E, Riessland M, Schreml J, Wirth B, Gillingwater TH, Parson SH.

Neurosci Lett. 2013 Jun 7;544:100-4. doi: 10.1016/j.neulet.2013.03.052. Epub 2013 Apr 10.


Chronic treatment with lithium does not improve neuromuscular phenotype in a mouse model of severe spinal muscular atrophy.

Dachs E, Piedrafita L, Hereu M, Esquerda JE, Calderó J.

Neuroscience. 2013 Oct 10;250:417-33. doi: 10.1016/j.neuroscience.2013.07.026. Epub 2013 Jul 19.


The Smn-independent beneficial effects of trichostatin A on an intermediate mouse model of spinal muscular atrophy.

Liu H, Yazdani A, Murray LM, Beauvais A, Kothary R.

PLoS One. 2014 Jul 1;9(7):e101225. doi: 10.1371/journal.pone.0101225. eCollection 2014.


SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy.

Riessland M, Ackermann B, Förster A, Jakubik M, Hauke J, Garbes L, Fritzsche I, Mende Y, Blumcke I, Hahnen E, Wirth B.

Hum Mol Genet. 2010 Apr 15;19(8):1492-506. doi: 10.1093/hmg/ddq023. Epub 2010 Jan 22.


Delivery of recombinant follistatin lessens disease severity in a mouse model of spinal muscular atrophy.

Rose FF Jr, Mattis VB, Rindt H, Lorson CL.

Hum Mol Genet. 2009 Mar 15;18(6):997-1005. doi: 10.1093/hmg/ddn426. Epub 2008 Dec 12.


Triptolide increases transcript and protein levels of survival motor neurons in human SMA fibroblasts and improves survival in SMA-like mice.

Hsu YY, Jong YJ, Tsai HH, Tseng YT, An LM, Lo YC.

Br J Pharmacol. 2012 Jun;166(3):1114-26. doi: 10.1111/j.1476-5381.2012.01829.x.


Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy.

Martinez TL, Kong L, Wang X, Osborne MA, Crowder ME, Van Meerbeke JP, Xu X, Davis C, Wooley J, Goldhamer DJ, Lutz CM, Rich MM, Sumner CJ.

J Neurosci. 2012 Jun 20;32(25):8703-15. doi: 10.1523/JNEUROSCI.0204-12.2012.


Chronic Treatment with the AMPK Agonist AICAR Prevents Skeletal Muscle Pathology but Fails to Improve Clinical Outcome in a Mouse Model of Severe Spinal Muscular Atrophy.

Cerveró C, Montull N, Tarabal O, Piedrafita L, Esquerda JE, Calderó J.

Neurotherapeutics. 2016 Jan;13(1):198-216. doi: 10.1007/s13311-015-0399-x.


Transgenic inactivation of murine myostatin does not decrease the severity of disease in a model of Spinal Muscular Atrophy.

Rindt H, Buckley DM, Vale SM, Krogman M, Rose FF Jr, Garcia ML, Lorson CL.

Neuromuscul Disord. 2012 Mar;22(3):277-85. doi: 10.1016/j.nmd.2011.10.012. Epub 2011 Nov 10.


The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells.

Riessland M, Brichta L, Hahnen E, Wirth B.

Hum Genet. 2006 Aug;120(1):101-10. Epub 2006 May 25.


Beta-lactam antibiotic offers neuroprotection in a spinal muscular atrophy model by multiple mechanisms.

Nizzardo M, Nardini M, Ronchi D, Salani S, Donadoni C, Fortunato F, Colciago G, Falcone M, Simone C, Riboldi G, Govoni A, Bresolin N, Comi GP, Corti S.

Exp Neurol. 2011 Jun;229(2):214-25. doi: 10.1016/j.expneurol.2011.01.017. Epub 2011 Feb 2.


Survival motor neuron gene 2 silencing by DNA methylation correlates with spinal muscular atrophy disease severity and can be bypassed by histone deacetylase inhibition.

Hauke J, Riessland M, Lunke S, Eyüpoglu IY, Blümcke I, El-Osta A, Wirth B, Hahnen E.

Hum Mol Genet. 2009 Jan 15;18(2):304-17. doi: 10.1093/hmg/ddn357. Epub 2008 Oct 29.


Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy.

Avila AM, Burnett BG, Taye AA, Gabanella F, Knight MA, Hartenstein P, Cizman Z, Di Prospero NA, Pellizzoni L, Fischbeck KH, Sumner CJ.

J Clin Invest. 2007 Mar;117(3):659-71. Epub 2007 Feb 22.

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