323 related articles for article (PubMed ID: 22701626)
1. 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]
2. 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]
3. 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]
4. 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]
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. 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]
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. PubMed ID: 23919525
[TBL] [Abstract][Full Text] [Related]
8. 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. PubMed ID: 24664736
[TBL] [Abstract][Full Text] [Related]
9. 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]
10. 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]
11. 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]
12. 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]
13. 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]
14. Further Characterization of the Predominant Inner Retinal Degeneration of Aging Cln3
Volz C; Mirza M; Langmann T; Jägle H
Adv Exp Med Biol; 2018; 1074():403-411. PubMed ID: 29721970
[TBL] [Abstract][Full Text] [Related]
15. Caspase 1 activity influences juvenile Batten disease (CLN3) pathogenesis.
Burkovetskaya M; Bosch ME; Karpuk N; Fallet R; Kielian T
J Neurochem; 2019 Mar; 148(5):652-668. PubMed ID: 29873075
[TBL] [Abstract][Full Text] [Related]
16. 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]
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. 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. 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]
20. 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]
[Next] [New Search]
