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5. 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]
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. 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]
8. 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]
9. 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]
10. 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]
11. 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]
12. Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender. Kovács AD; Pearce DA Dis Model Mech; 2015 Apr; 8(4):351-61. PubMed ID: 26035843 [TBL] [Abstract][Full Text] [Related]
13. Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis. Somogyi A; Petcherski A; Beckert B; Huebecker M; Priestman DA; Banning A; Cotman SL; Platt FM; Ruonala MO; Tikkanen R Int J Mol Sci; 2018 Feb; 19(2):. PubMed ID: 29470438 [TBL] [Abstract][Full Text] [Related]
14. 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]
15. 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]
16. 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; 5(1):74. PubMed ID: 29041969 [TBL] [Abstract][Full Text] [Related]
17. 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]
18. 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; 9(4):e95023. PubMed ID: 24736558 [TBL] [Abstract][Full Text] [Related]
19. 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]
20. 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] [Next] [New Search]