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321 related items for PubMed ID: 29964296

  • 1. Astrocytes in juvenile neuronal ceroid lipofuscinosis (CLN3) display metabolic and calcium signaling abnormalities.
    Bosch ME, Kielian T.
    J Neurochem; 2019 Mar; 148(5):612-624. PubMed ID: 29964296
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  • 3. Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3(Δex7/8)-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis.
    Finn R, Kovács AD, Pearce DA.
    Neurochem Int; 2011 May; 58(6):648-55. PubMed ID: 21315126
    [Abstract] [Full Text] [Related]

  • 4. Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro-inflammatory phenotype.
    Xiong J, Kielian T.
    J Neurochem; 2013 Oct; 127(2):245-58. PubMed ID: 23919525
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  • 6. Partial correction of the CNS lysosomal storage defect in a mouse model of juvenile neuronal ceroid lipofuscinosis by neonatal CNS administration of an adeno-associated virus serotype rh.10 vector expressing the human CLN3 gene.
    Sondhi D, Scott EC, Chen A, Hackett NR, Wong AM, Kubiak A, Nelvagal HR, Pearse Y, Cotman SL, Cooper JD, Crystal RG.
    Hum Gene Ther; 2014 Mar; 25(3):223-39. PubMed ID: 24372003
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  • 7. Age-dependent alterations in neuronal activity in the hippocampus and visual cortex in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis (CLN3).
    Burkovetskaya M, Karpuk N, Kielian T.
    Neurobiol Dis; 2017 Apr; 100():19-29. PubMed ID: 28042098
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  • 8. Caspase 1 activity influences juvenile Batten disease (CLN3) pathogenesis.
    Burkovetskaya M, Bosch ME, Karpuk N, Fallet R, Kielian T.
    J Neurochem; 2019 Mar; 148(5):652-668. PubMed ID: 29873075
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  • 9. Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL).
    Burkovetskaya M, Karpuk N, Xiong J, Bosch M, Boska MD, Takeuchi H, Suzumura A, Kielian T.
    PLoS One; 2014 Mar; 9(4):e95023. PubMed ID: 24736558
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  • 10. Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cells.
    Cao Y, Staropoli JF, Biswas S, Espinola JA, MacDonald ME, Lee JM, Cotman SL.
    PLoS One; 2011 Feb 17; 6(2):e17118. PubMed ID: 21359198
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  • 11. Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system.
    Staropoli JF, Haliw L, Biswas S, Garrett L, Hölter SM, Becker L, Skosyrski S, Da Silva-Buttkus P, Calzada-Wack J, Neff F, Rathkolb B, Rozman J, Schrewe A, Adler T, Puk O, Sun M, Favor J, Racz I, Bekeredjian R, Busch DH, Graw J, Klingenspor M, Klopstock T, Wolf E, Wurst W, Zimmer A, Lopez E, Harati H, Hill E, Krause DS, Guide J, Dragileva E, Gale E, Wheeler VC, Boustany RM, Brown DE, Breton S, Ruether K, Gailus-Durner V, Fuchs H, de Angelis MH, Cotman SL.
    PLoS One; 2012 Feb 17; 7(6):e38310. PubMed ID: 22701626
    [Abstract] [Full Text] [Related]

  • 12. Cln3(Deltaex7/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth.
    Cotman SL, Vrbanac V, Lebel LA, Lee RL, Johnson KA, Donahue LR, Teed AM, Antonellis K, Bronson RT, Lerner TJ, MacDonald ME.
    Hum Mol Genet; 2002 Oct 15; 11(22):2709-21. PubMed ID: 12374761
    [Abstract] [Full Text] [Related]

  • 13. Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome.
    Appu AP, Bagh MB, Sadhukhan T, Mondal A, Casey S, Mukherjee AB.
    J Inherit Metab Dis; 2019 Sep 15; 42(5):944-954. PubMed ID: 31025705
    [Abstract] [Full Text] [Related]

  • 14. Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons.
    Parviainen L, Dihanich S, Anderson GW, Wong AM, Brooks HR, Abeti R, Rezaie P, Lalli G, Pope S, Heales SJ, Mitchison HM, Williams BP, Cooper JD.
    Acta Neuropathol Commun; 2017 Oct 17; 5(1):74. PubMed ID: 29041969
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  • 15. Immunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage.
    Dannhausen K, Möhle C, Langmann T.
    Dis Model Mech; 2018 Sep 05; 11(9):. PubMed ID: 30042155
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  • 16. Neurodevelopmental delay in the Cln3Deltaex7/8 mouse model for Batten disease.
    Osório NS, Sampaio-Marques B, Chan CH, Oliveira P, Pearce DA, Sousa N, Rodrigues F.
    Genes Brain Behav; 2009 Apr 05; 8(3):337-45. PubMed ID: 19243453
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  • 17. Developmental impairments of select neurotransmitter systems in brains of Cln3(Deltaex7/8) knock-in mice, an animal model of juvenile neuronal ceroid lipofuscinosis.
    Herrmann P, Druckrey-Fiskaaen C, Kouznetsova E, Heinitz K, Bigl M, Cotman SL, Schliebs R.
    J Neurosci Res; 2008 Jun 05; 86(8):1857-70. PubMed ID: 18265413
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  • 18. Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium.
    Zhong Y, Mohan K, Liu J, Al-Attar A, Lin P, Flight RM, Sun Q, Warmoes MO, Deshpande RR, Liu H, Jung KS, Mitov MI, Lin N, Butterfield DA, Lu S, Liu J, Moseley HNB, Fan TWM, Kleinman ME, Wang QJ.
    Biochim Biophys Acta Mol Basis Dis; 2020 Oct 01; 1866(10):165883. PubMed ID: 32592935
    [Abstract] [Full Text] [Related]

  • 19. Retinal function in aging homozygous Cln3 (Δex7/8) knock-in mice.
    Volz C, Mirza M, Langmann T, Jägle H.
    Adv Exp Med Biol; 2014 Oct 01; 801():495-501. PubMed ID: 24664736
    [Abstract] [Full Text] [Related]

  • 20. Transcript and in silico analysis of CLN3 in juvenile neuronal ceroid lipofuscinosis and associated mouse models.
    Chan CH, Mitchison HM, Pearce DA.
    Hum Mol Genet; 2008 Nov 01; 17(21):3332-9. PubMed ID: 18678598
    [Abstract] [Full Text] [Related]

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