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Results: 1 to 20 of 119

1.

Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis.

Weimer JM, Benedict JW, Elshatory YM, Short DW, Ramirez-Montealegre D, Ryan DA, Alexander NA, Federoff HJ, Cooper JD, Pearce DA.

Brain Res. 2007 Aug 8;1162:98-112. Epub 2007 May 21.

PMID:
17617387
[PubMed - indexed for MEDLINE]
2.

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 17;1266:93-107. doi: 10.1016/j.brainres.2009.02.009. Epub 2009 Feb 20.

PMID:
19230832
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis.

Katz ML, Johnson GS, Tullis GE, Lei B.

Neurobiol Dis. 2008 Feb;29(2):242-53. Epub 2007 Sep 7.

PMID:
17962032
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

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 1;17(21):3332-9. doi: 10.1093/hmg/ddn228. Epub 2008 Aug 4.

PMID:
18678598
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Altered flurothyl seizure induction latency, phenotype, and subsequent mortality in a mouse model of juvenile neuronal ceroid lipofuscinosis/batten disease.

Kriscenski-Perry E, Applegate CD, Serour A, Mhyre TR, Leonardo CC, Pearce DA.

Epilepsia. 2002 Oct;43(10):1137-40.

PMID:
12366726
[PubMed - indexed for MEDLINE]
6.

Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).

Seigel GM, Lotery A, Kummer A, Bernard DJ, Greene ND, Turmaine M, Derksen T, Nussbaum RL, Davidson B, Wagner J, Mitchison HM.

Mol Cell Neurosci. 2002 Apr;19(4):515-27.

PMID:
11988019
[PubMed - indexed for MEDLINE]
7.

Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease.

Kovács AD, Saje A, Wong A, Ramji S, Cooper JD, Pearce DA.

Neuropharmacology. 2012 Oct;63(5):769-75. doi: 10.1016/j.neuropharm.2012.05.040. Epub 2012 Jun 6.

PMID:
22683643
[PubMed - indexed for MEDLINE]
Free PMC Article
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. doi: 10.1016/j.nbd.2010.09.011. Epub 2010 Sep 25.

PMID:
20875858
[PubMed - indexed for MEDLINE]
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. doi: 10.1371/journal.pone.0038310. Epub 2012 Jun 6.

PMID:
22701626
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease.

Pontikis CC, Cotman SL, MacDonald ME, Cooper JD.

Neurobiol Dis. 2005 Dec;20(3):823-36. Epub 2005 Jul 11.

PMID:
16006136
[PubMed - indexed for MEDLINE]
11.

Age-related decline in striatal dopamine content and motor performance occurs in the absence of nigral cell loss in a genetic mouse model of Parkinson's disease.

Colebrooke RE, Humby T, Lynch PJ, McGowan DP, Xia J, Emson PC.

Eur J Neurosci. 2006 Nov;24(9):2622-30.

PMID:
17100850
[PubMed - indexed for MEDLINE]
12.

Non-invasive assessment of retinal alterations in mouse models of infantile and juvenile neuronal ceroid lipofuscinosis by spectral domain optical coherence tomography.

Groh J, Stadler D, Buttmann M, Martini R.

Acta Neuropathol Commun. 2014 May 10;2:54. doi: 10.1186/2051-5960-2-54.

PMID:
24887158
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

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. doi: 10.1111/j.1601-183X.2009.00478.x. Epub 2009 Feb 19.

PMID:
19243453
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

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. doi: 10.1089/hum.2012.253. Epub 2014 Mar 4.

PMID:
24372003
[PubMed - indexed for MEDLINE]
15.

Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.

Pontikis CC, Cella CV, Parihar N, Lim MJ, Chakrabarti S, Mitchison HM, Mobley WC, Rezaie P, Pearce DA, Cooper JD.

Brain Res. 2004 Oct 15;1023(2):231-42.

PMID:
15374749
[PubMed - indexed for MEDLINE]
16.

Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.

Luiro K, Kopra O, Blom T, Gentile M, Mitchison HM, Hovatta I, Törnquist K, Jalanko A.

J Neurosci Res. 2006 Oct;84(5):1124-38.

PMID:
16941499
[PubMed - indexed for MEDLINE]
17.

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].

Mitchison HM, Bernard DJ, Greene ND, Cooper JD, Junaid MA, Pullarkat RK, de Vos N, Breuning MH, Owens JW, Mobley WC, Gardiner RM, Lake BD, Taschner PE, Nussbaum RL.

Neurobiol Dis. 1999 Oct;6(5):321-34. Erratum in: Neurobiol Dis 2000 Apr;7(2):127.

PMID:
10527801
[PubMed - indexed for MEDLINE]
18.

Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.

Weimer JM, Custer AW, Benedict JW, Alexander NA, Kingsley E, Federoff HJ, Cooper JD, Pearce DA.

Neurobiol Dis. 2006 May;22(2):284-93. Epub 2006 Jan 18.

PMID:
16412658
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

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. doi: 10.1111/jnc.12385. Epub 2013 Aug 22.

PMID:
23919525
[PubMed - indexed for MEDLINE]
20.

Progressive oxidative damage in the central nervous system of a murine model for juvenile Batten disease.

Benedict JW, Sommers CA, Pearce DA.

J Neurosci Res. 2007 Oct;85(13):2882-91.

PMID:
17638298
[PubMed - indexed for MEDLINE]

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