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Journal Abstract Search

267 related items for PubMed ID: 15193291

  • 1. Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis.
    Bible E, Gupta P, Hofmann SL, Cooper JD.
    Neurobiol Dis; 2004 Jul; 16(2):346-59. PubMed ID: 15193291
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  • 2. Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis.
    Griffey M, Bible E, Vogler C, Levy B, Gupta P, Cooper J, Sands MS.
    Neurobiol Dis; 2004 Jul; 16(2):360-9. PubMed ID: 15193292
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  • 5. Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.
    Kielar C, Maddox L, Bible E, Pontikis CC, Macauley SL, Griffey MA, Wong M, Sands MS, Cooper JD.
    Neurobiol Dis; 2007 Jan; 25(1):150-62. PubMed ID: 17046272
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  • 6. Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.
    Pontikis CC, Cella CV, Parihar N, Lim MJ, Chakrabarti S, Mitchison HM, Mobley WC, Rezaie P, Pearce DA, Cooper JD.
    Brain Res; 2004 Oct 15; 1023(2):231-42. PubMed ID: 15374749
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  • 7. Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.
    Groh J, Kühl TG, Ip CW, Nelvagal HR, Sri S, Duckett S, Mirza M, Langmann T, Cooper JD, Martini R.
    Brain; 2013 Apr 15; 136(Pt 4):1083-101. PubMed ID: 23485853
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  • 9. Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction.
    Ahtiainen L, Kolikova J, Mutka AL, Luiro K, Gentile M, Ikonen E, Khiroug L, Jalanko A, Kopra O.
    Neurobiol Dis; 2007 Oct 15; 28(1):52-64. PubMed ID: 17656100
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  • 10. AAV2-mediated ocular gene therapy for infantile neuronal ceroid lipofuscinosis.
    Griffey M, Macauley SL, Ogilvie JM, Sands MS.
    Mol Ther; 2005 Sep 15; 12(3):413-21. PubMed ID: 15979943
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  • 11. Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis.
    Lange J, Haslett LJ, Lloyd-Evans E, Pocock JM, Sands MS, Williams BP, Cooper JD.
    Acta Neuropathol Commun; 2018 Aug 08; 6(1):74. PubMed ID: 30089511
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  • 12. Novel neuroimaging finding in palmitoyl protein thioesterase-1-related neuronal ceroid lipofuscinosis.
    Kamate M, Hattiholi V.
    Pediatr Neurol; 2012 May 08; 46(5):325-8. PubMed ID: 22520356
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  • 13. Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis.
    Tamaki SJ, Jacobs Y, Dohse M, Capela A, Cooper JD, Reitsma M, He D, Tushinski R, Belichenko PV, Salehi A, Mobley W, Gage FH, Huhn S, Tsukamoto AS, Weissman IL, Uchida N.
    Cell Stem Cell; 2009 Sep 04; 5(3):310-9. PubMed ID: 19733542
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  • 14. The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis.
    Macauley SL, Pekny M, Sands MS.
    J Neurosci; 2011 Oct 26; 31(43):15575-85. PubMed ID: 22031903
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  • 15. Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?
    Shyng C, Sands MS.
    Biochem Soc Trans; 2014 Oct 26; 42(5):1282-5. PubMed ID: 25233404
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  • 16. Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.
    Lu JY, Nelvagal HR, Wang L, Birnbaum SG, Cooper JD, Hofmann SL.
    Mol Genet Metab; 2015 Oct 26; 116(1-2):98-105. PubMed ID: 25982063
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  • 17. Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze.
    Wendt KD, Lei B, Schachtman TR, Tullis GE, Ibe ME, Katz ML.
    Behav Brain Res; 2005 Jun 20; 161(2):175-82. PubMed ID: 15885820
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  • 18. Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis.
    Sarkar C, Sadhukhan T, Bagh MB, Appu AP, Chandra G, Mondal A, Saha A, Mukherjee AB.
    J Inherit Metab Dis; 2020 Sep 20; 43(5):1082-1101. PubMed ID: 32279353
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  • 19. Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice.
    Gupta P, Soyombo AA, Atashband A, Wisniewski KE, Shelton JM, Richardson JA, Hammer RE, Hofmann SL.
    Proc Natl Acad Sci U S A; 2001 Nov 20; 98(24):13566-71. PubMed ID: 11717424
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  • 20. An anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosis.
    Macauley SL, Wong AM, Shyng C, Augner DP, Dearborn JT, Pearse Y, Roberts MS, Fowler SC, Cooper JD, Watterson DM, Sands MS.
    J Neurosci; 2014 Sep 24; 34(39):13077-82. PubMed ID: 25253854
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