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Results: 1 to 20 of 134

1.

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 17;6(2):e17118. doi: 10.1371/journal.pone.0017118.

PMID:
21359198
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

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. doi: 10.1016/j.neuint.2011.02.003. Epub 2011 Feb 17.

PMID:
21315126
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis.

Fossale E, Wolf P, Espinola JA, Lubicz-Nawrocka T, Teed AM, Gao H, Rigamonti D, Cattaneo E, MacDonald ME, Cotman SL.

BMC Neurosci. 2004 Dec 10;5:57.

PMID:
15588329
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

Lithium rescues the impaired autophagy process in CbCln3(Δex7/8/Δex7/8) cerebellar cells and reduces neuronal vulnerability to cell death via IMPase inhibition.

Chang JW, Choi H, Cotman SL, Jung YK.

J Neurochem. 2011 Feb;116(4):659-68. doi: 10.1111/j.1471-4159.2010.07158.x. Epub 2011 Jan 19.

PMID:
21175620
[PubMed - indexed for MEDLINE]
5.

Protein product of CLN6 gene responsible for variant late-onset infantile neuronal ceroid lipofuscinosis interacts with CRMP-2.

Benedict JW, Getty AL, Wishart TM, Gillingwater TH, Pearce DA.

J Neurosci Res. 2009 Jul;87(9):2157-66. doi: 10.1002/jnr.22032.

PMID:
19235893
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis.

Cao Y, Espinola JA, Fossale E, Massey AC, Cuervo AM, MacDonald ME, Cotman SL.

J Biol Chem. 2006 Jul 21;281(29):20483-93. Epub 2006 May 19.

PMID:
16714284
[PubMed - indexed for MEDLINE]
Free Article
7.

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]
8.

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.

Thelen M, Damme M, Schweizer M, Hagel C, Wong AM, Cooper JD, Braulke T, Galliciotti G.

PLoS One. 2012;7(4):e35493. doi: 10.1371/journal.pone.0035493. Epub 2012 Apr 20. Erratum in: PLoS One. 2012;7(5): doi/10.1371/annotation/a4b06d46-8eb9-4d15-a15a-41bf4b5ccb8b. Daμμe, Markus [corrected to Damme, Markus]..

PMID:
22536393
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

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.

PMID:
12374761
[PubMed - indexed for MEDLINE]
Free Article
10.

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
11.

A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.

Morgan JP, Magee H, Wong A, Nelson T, Koch B, Cooper JD, Weimer JM.

PLoS One. 2013 Nov 1;8(11):e78694. doi: 10.1371/journal.pone.0078694. eCollection 2013.

PMID:
24223841
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

Apoptotic photoreceptor loss and altered expression of lysosomal proteins in the nclf mouse model of neuronal ceroid lipofuscinosis.

Bartsch U, Galliciotti G, Jofre GF, Jankowiak W, Hagel C, Braulke T.

Invest Ophthalmol Vis Sci. 2013 Oct 23;54(10):6952-9. doi: 10.1167/iovs.13-12945.

PMID:
24084090
[PubMed - indexed for MEDLINE]
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. doi: 10.1002/jnr.21630.

PMID:
18265413
[PubMed - indexed for MEDLINE]
14.

Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9.

Bronson RT, Donahue LR, Johnson KR, Tanner A, Lane PW, Faust JR.

Am J Med Genet. 1998 May 26;77(4):289-97.

PMID:
9600738
[PubMed - indexed for MEDLINE]
15.

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
16.

CLN3p impacts galactosylceramide transport, raft morphology, and lipid content.

Rusyn E, Mousallem T, Persaud-Sawin DA, Miller S, Boustany RM.

Pediatr Res. 2008 Jun;63(6):625-31. doi: 10.1203/PDR.0b013e31816fdc17.

PMID:
18317235
[PubMed - indexed for MEDLINE]
17.

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
18.

Retinal function in aging homozygous Cln3 (Δex7/8) knock-in mice.

Volz C, Mirza M, Langmann T, Jägle H.

Adv Exp Med Biol. 2014;801:495-501. doi: 10.1007/978-1-4614-3209-8_63.

PMID:
24664736
[PubMed - indexed for MEDLINE]
19.

The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein.

Wheeler RB, Sharp JD, Schultz RA, Joslin JM, Williams RE, Mole SE.

Am J Hum Genet. 2002 Feb;70(2):537-42. Epub 2001 Nov 27.

PMID:
11727201
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

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]

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