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437 related items for PubMed ID: 23029491

  • 1. Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy.
    Lee AJ, Awano T, Park GH, Monani UR.
    PLoS One; 2012; 7(9):e46353. PubMed ID: 23029491
    [Abstract] [Full Text] [Related]

  • 2. Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene.
    Park GH, Maeno-Hikichi Y, Awano T, Landmesser LT, Monani UR.
    J Neurosci; 2010 Sep 08; 30(36):12005-19. PubMed ID: 20826664
    [Abstract] [Full Text] [Related]

  • 3. Motor neuronal repletion of the NMJ organizer, Agrin, modulates the severity of the spinal muscular atrophy disease phenotype in model mice.
    Kim JK, Caine C, Awano T, Herbst R, Monani UR.
    Hum Mol Genet; 2017 Jul 01; 26(13):2377-2385. PubMed ID: 28379354
    [Abstract] [Full Text] [Related]

  • 4. 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, Lutz C, Lorson CL.
    Transgenic Res; 2013 Oct 01; 22(5):1029-36. PubMed ID: 23512182
    [Abstract] [Full Text] [Related]

  • 5. Motor transmission defects with sex differences in a new mouse model of mild spinal muscular atrophy.
    Deguise MO, De Repentigny Y, Tierney A, Beauvais A, Michaud J, Chehade L, Thabet M, Paul B, Reilly A, Gagnon S, Renaud JM, Kothary R.
    EBioMedicine; 2020 May 01; 55():102750. PubMed ID: 32339936
    [Abstract] [Full Text] [Related]

  • 6. 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. PubMed ID: 22723710
    [Abstract] [Full Text] [Related]

  • 7. 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 20; 22(3):263-76. PubMed ID: 22071333
    [Abstract] [Full Text] [Related]

  • 8. Muscle-specific SMN reduction reveals motor neuron-independent disease in spinal muscular atrophy models.
    Kim JK, Jha NN, Feng Z, Faleiro MR, Chiriboga CA, Wei-Lapierre L, Dirksen RT, Ko CP, Monani UR.
    J Clin Invest; 2020 Mar 02; 130(3):1271-1287. PubMed ID: 32039917
    [Abstract] [Full Text] [Related]

  • 9. Interaction between alpha-COP and SMN ameliorates disease phenotype in a mouse model of spinal muscular atrophy.
    Custer SK, Astroski JW, Li HX, Androphy EJ.
    Biochem Biophys Res Commun; 2019 Jun 25; 514(2):530-537. PubMed ID: 31060774
    [Abstract] [Full Text] [Related]

  • 10. Astrocytes influence the severity of spinal muscular atrophy.
    Rindt H, Feng Z, Mazzasette C, Glascock JJ, Valdivia D, Pyles N, Crawford TO, Swoboda KJ, Patitucci TN, Ebert AD, Sumner CJ, Ko CP, Lorson CL.
    Hum Mol Genet; 2015 Jul 15; 24(14):4094-102. PubMed ID: 25911676
    [Abstract] [Full Text] [Related]

  • 11. 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 05; 478(7367):123-6. PubMed ID: 21979052
    [Abstract] [Full Text] [Related]

  • 12. A spinal muscular atrophy modifier implicates the SMN protein in SNARE complex assembly at neuromuscular synapses.
    Kim JK, Jha NN, Awano T, Caine C, Gollapalli K, Welby E, Kim SS, Fuentes-Moliz A, Wang X, Feng Z, Sera F, Takeda T, Homma S, Ko CP, Tabares L, Ebert AD, Rich MM, Monani UR.
    Neuron; 2023 May 03; 111(9):1423-1439.e4. PubMed ID: 36863345
    [Abstract] [Full Text] [Related]

  • 13. Hypothermia improves disease manifestations in SMA mice via SMN augmentation.
    Tsai LK, Chen CL, Tsai YC, Ting CH, Chien YH, Lee NC, Hwu WL.
    Hum Mol Genet; 2016 Feb 15; 25(4):631-41. PubMed ID: 26647309
    [Abstract] [Full Text] [Related]

  • 14. 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 08; 12():25. PubMed ID: 21385431
    [Abstract] [Full Text] [Related]

  • 15. Histopathological Defects in Intestine in Severe Spinal Muscular Atrophy Mice Are Improved by Systemic Antisense Oligonucleotide Treatment.
    Sintusek P, Catapano F, Angkathunkayul N, Marrosu E, Parson SH, Morgan JE, Muntoni F, Zhou H.
    PLoS One; 2016 Mar 08; 11(5):e0155032. PubMed ID: 27163330
    [Abstract] [Full Text] [Related]

  • 16. Zebrafish survival motor neuron mutants exhibit presynaptic neuromuscular junction defects.
    Boon KL, Xiao S, McWhorter ML, Donn T, Wolf-Saxon E, Bohnsack MT, Moens CB, Beattie CE.
    Hum Mol Genet; 2009 Oct 01; 18(19):3615-25. PubMed ID: 19592581
    [Abstract] [Full Text] [Related]

  • 17. Plastin 3 Expression Does Not Modify Spinal Muscular Atrophy Severity in the ∆7 SMA Mouse.
    McGovern VL, Massoni-Laporte A, Wang X, Le TT, Le HT, Beattie CE, Rich MM, Burghes AH.
    PLoS One; 2015 Oct 01; 10(7):e0132364. PubMed ID: 26134627
    [Abstract] [Full Text] [Related]

  • 18. 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. PubMed ID: 20097677
    [Abstract] [Full Text] [Related]

  • 19. At the "junction" of spinal muscular atrophy pathogenesis: the role of neuromuscular junction dysfunction in SMA disease progression.
    Goulet BB, Kothary R, Parks RJ.
    Curr Mol Med; 2013 Aug 15; 13(7):1160-74. PubMed ID: 23514457
    [Abstract] [Full Text] [Related]

  • 20. AAV9-Mediated Expression of SMN Restricted to Neurons Does Not Rescue the Spinal Muscular Atrophy Phenotype in Mice.
    Besse A, Astord S, Marais T, Roda M, Giroux B, Lejeune FX, Relaix F, Smeriglio P, Barkats M, Biferi MG.
    Mol Ther; 2020 Aug 05; 28(8):1887-1901. PubMed ID: 32470325
    [Abstract] [Full Text] [Related]

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