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5. 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]
6. 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]
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. A knock-in reporter mouse model for Batten disease reveals predominant expression of Cln3 in visual, limbic and subcortical motor structures. Ding SL; Tecedor L; Stein CS; Davidson BL Neurobiol Dis; 2011 Feb; 41(2):237-48. PubMed ID: 20875858 [TBL] [Abstract][Full Text] [Related]
9. Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis. Oetjen S; Kuhl D; Hermey G J Neurochem; 2016 Nov; 139(3):456-470. PubMed ID: 27453211 [TBL] [Abstract][Full Text] [Related]
10. 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]
11. 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]
12. CLN3L, a novel protein related to the Batten disease protein, is overexpressed in Cln3-/- mice and in Batten disease. Narayan SB; Pastor JV; Mitchison HM; Bennett MJ Brain; 2004 Aug; 127(Pt 8):1748-54. PubMed ID: 15240430 [TBL] [Abstract][Full Text] [Related]
13. A function retained by the common mutant CLN3 protein is responsible for the late onset of juvenile neuronal ceroid lipofuscinosis. Kitzmüller C; Haines RL; Codlin S; Cutler DF; Mole SE Hum Mol Genet; 2008 Jan; 17(2):303-12. PubMed ID: 17947292 [TBL] [Abstract][Full Text] [Related]
16. Altered gene expression in the eye of a mouse model for batten disease. Chattopadhyay S; Kingsley E; Serour A; Curran TM; Brooks AI; Pearce DA Invest Ophthalmol Vis Sci; 2004 Sep; 45(9):2893-905. PubMed ID: 15326100 [TBL] [Abstract][Full Text] [Related]
17. A novel c.1135_1138delCTGT mutation in CLN3 leads to juvenile neuronal ceroid lipofuscinosis. Drack AV; Miller JN; Pearce DA J Child Neurol; 2013 Sep; 28(9):1112-6. PubMed ID: 23877479 [TBL] [Abstract][Full Text] [Related]
18. Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis. Weimer JM; Benedict JW; Getty AL; Pontikis CC; Lim MJ; Cooper JD; Pearce DA Brain Res; 2009 Apr; 1266():93-107. PubMed ID: 19230832 [TBL] [Abstract][Full Text] [Related]
19. Novel interactions of CLN3 protein link Batten disease to dysregulation of fodrin-Na+, K+ ATPase complex. Uusi-Rauva K; Luiro K; Tanhuanpää K; Kopra O; Martín-Vasallo P; Kyttälä A; Jalanko A Exp Cell Res; 2008 Sep; 314(15):2895-905. PubMed ID: 18621045 [TBL] [Abstract][Full Text] [Related]
20. 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] [Next] [New Search]