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Journal Abstract Search

397 related items for PubMed ID: 27170316

  • 1. Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy.
    Hunter G, Powis RA, Jones RA, Groen EJ, Shorrock HK, Lane FM, Zheng Y, Sherman DL, Brophy PJ, Gillingwater TH.
    Hum Mol Genet; 2016 Jul 01; 25(13):2853-2861. PubMed ID: 27170316
    [Abstract] [Full Text] [Related]

  • 2. SMN-dependent intrinsic defects in Schwann cells in mouse models of spinal muscular atrophy.
    Hunter G, Aghamaleky Sarvestany A, Roche SL, Symes RC, Gillingwater TH.
    Hum Mol Genet; 2014 May 01; 23(9):2235-50. PubMed ID: 24301677
    [Abstract] [Full Text] [Related]

  • 3. ZPR1 prevents R-loop accumulation, upregulates SMN2 expression and rescues spinal muscular atrophy.
    Kannan A, Jiang X, He L, Ahmad S, Gangwani L.
    Brain; 2020 Jan 01; 143(1):69-93. PubMed ID: 31828288
    [Abstract] [Full Text] [Related]

  • 4. 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]

  • 5. The zinc finger protein ZPR1 is a potential modifier of spinal muscular atrophy.
    Ahmad S, Wang Y, Shaik GM, Burghes AH, Gangwani L.
    Hum Mol Genet; 2012 Jun 15; 21(12):2745-58. PubMed ID: 22422766
    [Abstract] [Full Text] [Related]

  • 6. Normalization of Patient-Identified Plasma Biomarkers in SMNΔ7 Mice following Postnatal SMN Restoration.
    Arnold WD, Duque S, Iyer CC, Zaworski P, McGovern VL, Taylor SJ, von Herrmann KM, Kobayashi DT, Chen KS, Kolb SJ, Paushkin SV, Burghes AH.
    PLoS One; 2016 Jun 15; 11(12):e0167077. PubMed ID: 27907033
    [Abstract] [Full Text] [Related]

  • 7. 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]

  • 8. 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]

  • 9. 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 05; 22(3):263-76. PubMed ID: 22071333
    [Abstract] [Full Text] [Related]

  • 10. Notch Signaling Mediates Astrocyte Abnormality in Spinal Muscular Atrophy Model Systems.
    Ohuchi K, Funato M, Yoshino Y, Ando S, Inagaki S, Sato A, Kawase C, Seki J, Saito T, Nishio H, Nakamura S, Shimazawa M, Kaneko H, Hara H.
    Sci Rep; 2019 Mar 06; 9(1):3701. PubMed ID: 30842449
    [Abstract] [Full Text] [Related]

  • 11. 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 06; 22(5):1029-36. PubMed ID: 23512182
    [Abstract] [Full Text] [Related]

  • 12. 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 06; 55():102750. PubMed ID: 32339936
    [Abstract] [Full Text] [Related]

  • 13. 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 01; 23(7):1754-70. PubMed ID: 24218366
    [Abstract] [Full Text] [Related]

  • 14. 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]

  • 15. 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. PubMed ID: 19329542
    [Abstract] [Full Text] [Related]

  • 16. The Antisense Transcript SMN-AS1 Regulates SMN Expression and Is a Novel Therapeutic Target for Spinal Muscular Atrophy.
    d'Ydewalle C, Ramos DM, Pyles NJ, Ng SY, Gorz M, Pilato CM, Ling K, Kong L, Ward AJ, Rubin LL, Rigo F, Bennett CF, Sumner CJ.
    Neuron; 2017 Jan 04; 93(1):66-79. PubMed ID: 28017471
    [Abstract] [Full Text] [Related]

  • 17. 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]

  • 18. Oligodendrocyte development and CNS myelination are unaffected in a mouse model of severe spinal muscular atrophy.
    O'Meara RW, Cummings SE, De Repentigny Y, McFall E, Michalski JP, Deguise MO, Gibeault S, Kothary R.
    Hum Mol Genet; 2017 Jan 15; 26(2):282-292. PubMed ID: 28069797
    [Abstract] [Full Text] [Related]

  • 19. Defects in neuromuscular junction remodelling in the Smn(2B/-) mouse model of spinal muscular atrophy.
    Murray LM, Beauvais A, Bhanot K, Kothary R.
    Neurobiol Dis; 2013 Jan 15; 49():57-67. PubMed ID: 22960106
    [Abstract] [Full Text] [Related]

  • 20. 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]

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