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Journal Abstract Search

385 related items for PubMed ID: 23919525

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  • 2. 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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  • 3. Self-Complementary AAV9 Gene Delivery Partially Corrects Pathology Associated with Juvenile Neuronal Ceroid Lipofuscinosis (CLN3).
    Bosch ME, Aldrich A, Fallet R, Odvody J, Burkovetskaya M, Schuberth K, Fitzgerald JA, Foust KD, Kielian T.
    J Neurosci; 2016 Sep 14; 36(37):9669-82. PubMed ID: 27629717
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  • 4. 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 14; 58(6):648-55. PubMed ID: 21315126
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  • 6. Astrocytes in juvenile neuronal ceroid lipofuscinosis (CLN3) display metabolic and calcium signaling abnormalities.
    Bosch ME, Kielian T.
    J Neurochem; 2019 Mar 14; 148(5):612-624. PubMed ID: 29964296
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  • 9. 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 14; 25(3):223-39. PubMed ID: 24372003
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  • 10. 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 14; 86(8):1857-70. PubMed ID: 18265413
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  • 12. 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 14; 42(5):944-954. PubMed ID: 31025705
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  • 13. 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
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  • 17. Activation of PPARα Exhibits Therapeutic Efficacy in a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis.
    Jana M, Dutta D, Poddar J, Pahan K.
    J Neurosci; 2023 Mar 08; 43(10):1814-1829. PubMed ID: 36697260
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  • 20. Protracted course of juvenile ceroid lipofuscinosis associated with a novel CLN3 mutation (p.Y199X).
    Sarpong A, Schottmann G, Rüther K, Stoltenburg G, Kohlschütter A, Hübner C, Schuelke M.
    Clin Genet; 2009 Jul 08; 76(1):38-45. PubMed ID: 19489875
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