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Items: 1 to 20 of 185

1.

Co-regulation of survival of motor neuron and Bcl-xL expression: implications for neuroprotection in spinal muscular atrophy.

Anderton RS, Price LL, Turner BJ, Meloni BP, Mitrpant C, Mastaglia FL, Goh C, Wilton SD, Boulos S.

Neuroscience. 2012 Sep 18;220:228-36. doi: 10.1016/j.neuroscience.2012.06.042. Epub 2012 Jun 23.

PMID:
22732506
2.

A new model to study spinal muscular atrophy: neurite degeneration and cell death is counteracted by BCL-X(L) Overexpression in motoneurons.

Garcera A, Mincheva S, Gou-Fabregas M, Caraballo-Miralles V, Lladó J, Comella JX, Soler RM.

Neurobiol Dis. 2011 Jun;42(3):415-26. doi: 10.1016/j.nbd.2011.02.003. Epub 2011 Feb 16.

PMID:
21333739
3.

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. Epub 2005 Feb 9.

PMID:
15703193
4.

Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?

Rossoll W, Kröning AK, Ohndorf UM, Steegborn C, Jablonka S, Sendtner M.

Hum Mol Genet. 2002 Jan 1;11(1):93-105.

PMID:
11773003
5.

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.

6.

Regulation of murine survival motor neuron (Smn) protein levels by modifying Smn exon 7 splicing.

DiDonato CJ, Lorson CL, De Repentigny Y, Simard L, Chartrand C, Androphy EJ, Kothary R.

Hum Mol Genet. 2001 Nov 1;10(23):2727-36.

PMID:
11726560
7.

Restoring Bcl-x(L) levels benefits a mouse model of spinal muscular atrophy.

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

Neurobiol Dis. 2008 Sep;31(3):361-7. doi: 10.1016/j.nbd.2008.05.014. Epub 2008 Jun 3.

PMID:
18590823
8.

Survival of motor neuron protein over-expression prevents calpain-mediated cleavage and activation of procaspase-3 in differentiated human SH-SY5Y cells.

Anderton RS, Meloni BP, Mastaglia FL, Greene WK, Boulos S.

Neuroscience. 2011 May 5;181:226-33. doi: 10.1016/j.neuroscience.2011.02.032. Epub 2011 Feb 17.

PMID:
21333717
9.

Hydroxyurea enhances SMN2 gene expression in spinal muscular atrophy cells.

Grzeschik SM, Ganta M, Prior TW, Heavlin WD, Wang CH.

Ann Neurol. 2005 Aug;58(2):194-202.

PMID:
16049920
10.

In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein.

Piazzon N, Rage F, Schlotter F, Moine H, Branlant C, Massenet S.

J Biol Chem. 2008 Feb 29;283(9):5598-610. Epub 2007 Dec 19.

11.

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.

PMID:
11734549
12.

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.

PMID:
19642194
13.

Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts.

Mattis VB, Rai R, Wang J, Chang CW, Coady T, Lorson CL.

Hum Genet. 2006 Nov;120(4):589-601. Epub 2006 Sep 2.

PMID:
16951947
14.

Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins.

Huo Q, Kayikci M, Odermatt P, Meyer K, Michels O, Saxena S, Ule J, Schümperli D.

RNA Biol. 2014;11(11):1430-46. doi: 10.1080/15476286.2014.996494.

15.

SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1.

Young PJ, DiDonato CJ, Hu D, Kothary R, Androphy EJ, Lorson CL.

Hum Mol Genet. 2002 Mar 1;11(5):577-87.

PMID:
11875052
16.

Delivery of bifunctional RNAs that target an intronic repressor and increase SMN levels in an animal model of spinal muscular atrophy.

Baughan TD, Dickson A, Osman EY, Lorson CL.

Hum Mol Genet. 2009 May 1;18(9):1600-11. doi: 10.1093/hmg/ddp076. Epub 2009 Feb 19.

17.

A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells.

Sharma A, Lambrechts A, Hao le T, Le TT, Sewry CA, Ampe C, Burghes AH, Morris GE.

Exp Cell Res. 2005 Sep 10;309(1):185-97.

PMID:
15975577
18.

Co-regulation of survival of motor neuron (SMN) protein and its interactor SIP1 during development and in spinal muscular atrophy.

Jablonka S, Bandilla M, Wiese S, Bühler D, Wirth B, Sendtner M, Fischer U.

Hum Mol Genet. 2001 Mar 1;10(5):497-505.

PMID:
11181573
19.

Synergistic anti-apoptotic activity between Bcl-2 and SMN implicated in spinal muscular atrophy.

Iwahashi H, Eguchi Y, Yasuhara N, Hanafusa T, Matsuzawa Y, Tsujimoto Y.

Nature. 1997 Nov 27;390(6658):413-7.

PMID:
9389483
20.

Proteomic assessment of a cell model of spinal muscular atrophy.

Wu CY, Whye D, Glazewski L, Choe L, Kerr D, Lee KH, Mason RW, Wang W.

BMC Neurosci. 2011 Mar 8;12:25. doi: 10.1186/1471-2202-12-25.

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