360 related articles for article (PubMed ID: 24372003)
1. 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
[TBL] [Abstract][Full Text] [Related]
2. 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; 36(37):9669-82. PubMed ID: 27629717
[TBL] [Abstract][Full Text] [Related]
3. 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; 42(5):944-954. PubMed ID: 31025705
[TBL] [Abstract][Full Text] [Related]
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; 58(6):648-55. PubMed ID: 21315126
[TBL] [Abstract][Full Text] [Related]
5. Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3).
Aldrich A; Bosch ME; Fallet R; Odvody J; Burkovetskaya M; Rama Rao KV; Cooper JD; Drack AV; Kielian T
Ann Neurol; 2016 Dec; 80(6):909-923. PubMed ID: 27804148
[TBL] [Abstract][Full Text] [Related]
6. 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
[TBL] [Abstract][Full Text] [Related]
7. 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; 43(10):1814-1829. PubMed ID: 36697260
[TBL] [Abstract][Full Text] [Related]
8. 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
[TBL] [Abstract][Full Text] [Related]
9. 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; 7(6):e38310. PubMed ID: 22701626
[TBL] [Abstract][Full Text] [Related]
10. 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
[TBL] [Abstract][Full Text] [Related]
11. 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; 17(21):3332-9. PubMed ID: 18678598
[TBL] [Abstract][Full Text] [Related]
12. Survival advantage of neonatal CNS gene transfer for late infantile neuronal ceroid lipofuscinosis.
Sondhi D; Peterson DA; Edelstein AM; del Fierro K; Hackett NR; Crystal RG
Exp Neurol; 2008 Sep; 213(1):18-27. PubMed ID: 18639872
[TBL] [Abstract][Full Text] [Related]
13. 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; 86(8):1857-70. PubMed ID: 18265413
[TBL] [Abstract][Full Text] [Related]
14. Long-term expression and safety of administration of AAVrh.10hCLN2 to the brain of rats and nonhuman primates for the treatment of late infantile neuronal ceroid lipofuscinosis.
Sondhi D; Johnson L; Purpura K; Monette S; Souweidane MM; Kaplitt MG; Kosofsky B; Yohay K; Ballon D; Dyke J; Kaminksy SM; Hackett NR; Crystal RG
Hum Gene Ther Methods; 2012 Oct; 23(5):324-35. PubMed ID: 23131032
[TBL] [Abstract][Full Text] [Related]
15. 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; 11(22):2709-21. PubMed ID: 12374761
[TBL] [Abstract][Full Text] [Related]
16. 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; 11(9):. PubMed ID: 30042155
[TBL] [Abstract][Full Text] [Related]
17. 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; 6(2):e17118. PubMed ID: 21359198
[TBL] [Abstract][Full Text] [Related]
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; 1866(10):165883. PubMed ID: 32592935
[TBL] [Abstract][Full Text] [Related]
19. Deletion of the Caenorhabditis elegans homologues of the CLN3 gene, involved in human juvenile neuronal ceroid lipofuscinosis, causes a mild progeric phenotype.
de Voer G; van der Bent P; Rodrigues AJ; van Ommen GJ; Peters DJ; Taschner PE
J Inherit Metab Dis; 2005; 28(6):1065-80. PubMed ID: 16435200
[TBL] [Abstract][Full Text] [Related]
20. 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; 8(3):337-45. PubMed ID: 19243453
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]