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Items: 1 to 20 of 258


Celecoxib increases SMN and survival in a severe spinal muscular atrophy mouse model via p38 pathway activation.

Farooq F, Abadía-Molina F, MacKenzie D, Hadwen J, Shamim F, O'Reilly S, Holcik M, MacKenzie A.

Hum Mol Genet. 2013 Sep 1;22(17):3415-24. doi: 10.1093/hmg/ddt191.


p38 Mitogen-activated protein kinase stabilizes SMN mRNA through RNA binding protein HuR.

Farooq F, Balabanian S, Liu X, Holcik M, MacKenzie A.

Hum Mol Genet. 2009 Nov 1;18(21):4035-45. doi: 10.1093/hmg/ddp352.


Prolactin increases SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway.

Farooq F, Molina FA, Hadwen J, MacKenzie D, Witherspoon L, Osmond M, Holcik M, MacKenzie A.

J Clin Invest. 2011 Aug;121(8):3042-50. doi: 10.1172/JCI46276. Erratum in: J Clin Invest. 2011 Sep 1;121(9):3763.


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.


Synthesis and biological evaluation of novel 2,4-diaminoquinazoline derivatives as SMN2 promoter activators for the potential treatment of spinal muscular atrophy.

Thurmond J, Butchbach ME, Palomo M, Pease B, Rao M, Bedell L, Keyvan M, Pai G, Mishra R, Haraldsson M, Andresson T, Bragason G, Thosteinsdottir M, Bjornsson JM, Coovert DD, Burghes AH, Gurney ME, Singh J.

J Med Chem. 2008 Feb 14;51(3):449-69. doi: 10.1021/jm061475p.


Shift from extracellular signal-regulated kinase to AKT/cAMP response element-binding protein pathway increases survival-motor-neuron expression in spinal-muscular-atrophy-like mice and patient cells.

Branchu J, Biondi O, Chali F, Collin T, Leroy F, Mamchaoui K, Makoukji J, Pariset C, Lopes P, Massaad C, Chanoine C, Charbonnier F.

J Neurosci. 2013 Mar 6;33(10):4280-94. doi: 10.1523/JNEUROSCI.2728-12.2013.


Development and characterization of an SMN2-based intermediate mouse model of Spinal Muscular Atrophy.

Cobb MS, Rose FF, Rindt H, Glascock JJ, Shababi M, Miller MR, Osman EY, Yen PF, Garcia ML, Martin BR, Wetz MJ, Mazzasette C, Feng Z, Ko CP, Lorson CL.

Hum Mol Genet. 2013 May 1;22(9):1843-55. doi: 10.1093/hmg/ddt037.


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.


A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice.

Workman E, Saieva L, Carrel TL, Crawford TO, Liu D, Lutz C, Beattie CE, Pellizzoni L, Burghes AH.

Hum Mol Genet. 2009 Jun 15;18(12):2215-29. doi: 10.1093/hmg/ddp157.


Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy.

Liu H, Shafey D, Moores JN, Kothary R.

J Neurosci Res. 2010 Jan;88(1):111-22. doi: 10.1002/jnr.22189.


Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model.

Hua Y, Sahashi K, Rigo F, Hung G, Horev G, Bennett CF, Krainer AR.

Nature. 2011 Oct 5;478(7367):123-6. doi: 10.1038/nature10485.


Aclarubicin treatment restores SMN levels to cells derived from type I spinal muscular atrophy patients.

Andreassi C, Jarecki J, Zhou J, Coovert DD, Monani UR, Chen X, Whitney M, Pollok B, Zhang M, Androphy E, Burghes AH.

Hum Mol Genet. 2001 Nov 15;10(24):2841-9.


SMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy.

See K, Yadav P, Giegerich M, Cheong PS, Graf M, Vyas H, Lee SG, Mathavan S, Fischer U, Sendtner M, Winkler C.

Hum Mol Genet. 2014 Apr 1;23(7):1754-70. doi: 10.1093/hmg/ddt567.


In vivo NMDA receptor activation accelerates motor unit maturation, protects spinal motor neurons, and enhances SMN2 gene expression in severe spinal muscular atrophy mice.

Biondi O, Branchu J, Sanchez G, Lancelin C, Deforges S, Lopes P, Pariset C, Lécolle S, Côté J, Chanoine C, Charbonnier F.

J Neurosci. 2010 Aug 25;30(34):11288-99. doi: 10.1523/JNEUROSCI.1764-10.2010.


Oligonucleotide-mediated survival of motor neuron protein expression in CNS improves phenotype in a mouse model of spinal muscular atrophy.

Williams JH, Schray RC, Patterson CA, Ayitey SO, Tallent MK, Lutz GJ.

J Neurosci. 2009 Jun 17;29(24):7633-8. doi: 10.1523/JNEUROSCI.0950-09.2009.


A paucisymptomatic neuromuscular disease mimicking type III 5q-SMA with complex rearrangements in the SMN gene.

Lohkamp LN, von Au K, Goebel HH, Kress W, Grieben U, Drossel K, Garbes L, Wirth B, Heppner FL, Stenzel W.

J Child Neurol. 2014 Feb;29(2):254-9. doi: 10.1177/0883073813511858.


Established Stem Cell Model of Spinal Muscular Atrophy Is Applicable in the Evaluation of the Efficacy of Thyrotropin-Releasing Hormone Analog.

Ohuchi K, Funato M, Kato Z, Seki J, Kawase C, Tamai Y, Ono Y, Nagahara Y, Noda Y, Kameyama T, Ando S, Tsuruma K, Shimazawa M, Hara H, Kaneko H.

Stem Cells Transl Med. 2016 Feb;5(2):152-63. doi: 10.5966/sctm.2015-0059.


SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN.

Le TT, Pham LT, Butchbach ME, Zhang HL, Monani UR, Coovert DD, Gavrilina TO, Xing L, Bassell GJ, Burghes AH.

Hum Mol Genet. 2005 Mar 15;14(6):845-57.


Restoration of SMN to Emx-1 expressing cortical neurons is not sufficient to provide benefit to a severe mouse model of Spinal Muscular Atrophy.

Taylor AS, Glascock JJ, Rose FF Jr, Lutz C, Lorson CL.

Transgenic Res. 2013 Oct;22(5):1029-36. doi: 10.1007/s11248-013-9702-y.

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