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401 related items for PubMed ID: 12111848

  • 1. Establishment and characterization of spontaneously immortalized Schwann cells from murine model of globoid cell leukodystrophy (twitcher).
    Shen JS, Watabe K, Meng XL, Ida H, Ohashi T, Eto Y.
    J Neurosci Res; 2002 Jun 01; 68(5):588-94. PubMed ID: 12111848
    [Abstract] [Full Text] [Related]

  • 2. Suppression of galactosylceramidase (GALC) expression in the twitcher mouse model of globoid cell leukodystrophy (GLD) is caused by nonsense-mediated mRNA decay (NMD).
    Lee WC, Tsoi YK, Dickey CA, Delucia MW, Dickson DW, Eckman CB.
    Neurobiol Dis; 2006 Aug 01; 23(2):273-80. PubMed ID: 16759875
    [Abstract] [Full Text] [Related]

  • 3. GALC transduction leads to morphological improvement of the twitcher oligodendrocytes in vivo.
    Meng XL, Shen JS, Watabe K, Ohashi T, Eto Y.
    Mol Genet Metab; 2005 Apr 01; 84(4):332-43. PubMed ID: 15781194
    [Abstract] [Full Text] [Related]

  • 4. Enzyme replacement therapy of a novel humanized mouse model of globoid cell leukodystrophy.
    Matthes F, Andersson C, Stein A, Eistrup C, Fogh J, Gieselmann V, Wenger DA, Matzner U.
    Exp Neurol; 2015 Sep 01; 271():36-45. PubMed ID: 25956830
    [Abstract] [Full Text] [Related]

  • 5. Generation of a mouse with low galactocerebrosidase activity by gene targeting: a new model of globoid cell leukodystrophy (Krabbe disease).
    Luzi P, Rafi MA, Zaka M, Curtis M, Vanier MT, Wenger DA.
    Mol Genet Metab; 2001 Jul 01; 73(3):211-23. PubMed ID: 11461188
    [Abstract] [Full Text] [Related]

  • 6. Murine model of genetic demyelinating disease: the twitcher mouse.
    Suzuki K, Taniike M.
    Microsc Res Tech; 1995 Oct 15; 32(3):204-14. PubMed ID: 8527855
    [Abstract] [Full Text] [Related]

  • 7. Intraventricular administration of recombinant adenovirus to neonatal twitcher mouse leads to clinicopathological improvements.
    Shen JS, Watabe K, Ohashi T, Eto Y.
    Gene Ther; 2001 Jul 15; 8(14):1081-7. PubMed ID: 11526455
    [Abstract] [Full Text] [Related]

  • 8. Intrinsic resistance of neural stem cells to toxic metabolites may make them well suited for cell non-autonomous disorders: evidence from a mouse model of Krabbe leukodystrophy.
    Taylor RM, Lee JP, Palacino JJ, Bower KA, Li J, Vanier MT, Wenger DA, Sidman RL, Snyder EY.
    J Neurochem; 2006 Jun 15; 97(6):1585-99. PubMed ID: 16805770
    [Abstract] [Full Text] [Related]

  • 9. AAV2/5 vector expressing galactocerebrosidase ameliorates CNS disease in the murine model of globoid-cell leukodystrophy more efficiently than AAV2.
    Lin D, Fantz CR, Levy B, Rafi MA, Vogler C, Wenger DA, Sands MS.
    Mol Ther; 2005 Sep 15; 12(3):422-30. PubMed ID: 15996520
    [Abstract] [Full Text] [Related]

  • 10. Mechanism-based combination treatment dramatically increases therapeutic efficacy in murine globoid cell leukodystrophy.
    Hawkins-Salsbury JA, Shea L, Jiang X, Hunter DA, Guzman AM, Reddy AS, Qin EY, Li Y, Gray SJ, Ory DS, Sands MS.
    J Neurosci; 2015 Apr 22; 35(16):6495-505. PubMed ID: 25904800
    [Abstract] [Full Text] [Related]

  • 11. Characterization and application of a disease-cell model for a neurodegenerative lysosomal disease.
    Ribbens JJ, Moser AB, Hubbard WC, Bongarzone ER, Maegawa GH.
    Mol Genet Metab; 2014 Feb 22; 111(2):172-83. PubMed ID: 24094551
    [Abstract] [Full Text] [Related]

  • 12. Galactosylceramidase deficiency causes sperm abnormalities in the mouse model of globoid cell leukodystrophy.
    Luddi A, Strazza M, Carbone M, Moretti E, Costantino-Ceccarini E.
    Exp Cell Res; 2005 Mar 10; 304(1):59-68. PubMed ID: 15707574
    [Abstract] [Full Text] [Related]

  • 13. Mitigation of cerebellar neuropathy in globoid cell leukodystrophy mice by AAV-mediated gene therapy.
    Lin DS, Hsiao CD, Lee AY, Ho CS, Liu HL, Wang TJ, Jian YR, Hsu JC, Huang ZD, Lee TH, Chiang MF.
    Gene; 2015 Oct 15; 571(1):81-90. PubMed ID: 26115766
    [Abstract] [Full Text] [Related]

  • 14. Peripheral neuropathy in the twitcher mouse: accumulation of extracellular matrix in the endoneurium and aberrant expression of ion channels.
    Kagitani-Shimono K, Mohri I, Yagi T, Taniike M, Suzuki K.
    Acta Neuropathol; 2008 May 15; 115(5):577-87. PubMed ID: 18172657
    [Abstract] [Full Text] [Related]

  • 15. CNS-targeted AAV5 gene transfer results in global dispersal of vector and prevention of morphological and function deterioration in CNS of globoid cell leukodystrophy mouse model.
    Lin DS, Hsiao CD, Liau I, Lin SP, Chiang MF, Chuang CK, Wang TJ, Wu TY, Jian YR, Huang SF, Liu HL.
    Mol Genet Metab; 2011 Aug 15; 103(4):367-77. PubMed ID: 21620749
    [Abstract] [Full Text] [Related]

  • 16. AAV-mediated expression of galactocerebrosidase in brain results in attenuated symptoms and extended life span in murine models of globoid cell leukodystrophy.
    Rafi MA, Zhi Rao H, Passini MA, Curtis M, Vanier MT, Zaka M, Luzi P, Wolfe JH, Wenger DA.
    Mol Ther; 2005 May 15; 11(5):734-44. PubMed ID: 15851012
    [Abstract] [Full Text] [Related]

  • 17. Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosides.
    Ichioka T, Kishimoto Y, Brennan S, Santos GW, Yeager AM.
    Proc Natl Acad Sci U S A; 1987 Jun 15; 84(12):4259-63. PubMed ID: 2884662
    [Abstract] [Full Text] [Related]

  • 18. Somatic cell genetic analysis of the galactocerebrosidase gene: lack of complementation in human Krabbe disease/twitcher mouse cell hybrids.
    Skiba MC, Lyerla TA, Konola JT, Raghavan S.
    J Neurosci Res; 1990 Dec 15; 27(4):472-8. PubMed ID: 2079710
    [Abstract] [Full Text] [Related]

  • 19. Sphingolipid profile in the CNS of the twitcher (globoid cell leukodystrophy) mouse: a lipidomics approach.
    Esch SW, Williams TD, Biswas S, Chakrabarty A, LeVine SM.
    Cell Mol Biol (Noisy-le-grand); 2003 Jul 15; 49(5):779-87. PubMed ID: 14528915
    [Abstract] [Full Text] [Related]

  • 20. Transplantation of mouse embryonic stem cell-derived oligodendrocytes in the murine model of globoid cell leukodystrophy.
    Kuai XL, Ni RZ, Zhou GX, Mao ZB, Zhang JF, Yi N, Liu ZX, Shao N, Ni WK, Wang ZW.
    Stem Cell Res Ther; 2015 Mar 14; 6(1):30. PubMed ID: 25888852
    [Abstract] [Full Text] [Related]

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