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Items: 1 to 20 of 101

1.

Donepezil enhances Purkinje cell survival and alleviates motor dysfunction by inhibiting cholesterol synthesis in a murine model of Niemann Pick disease type C.

Seo Y, Shin Y, Kim HS, Kang I, Hong IS, Choi SW, Yu KR, Kang KS.

J Neuropathol Exp Neurol. 2014 Mar;73(3):234-43. doi: 10.1097/NEN.0000000000000045.

2.

Human umbilical cord blood-derived mesenchymal stem cells protect against neuronal cell death and ameliorate motor deficits in Niemann Pick type C1 mice.

Seo Y, Yang SR, Jee MK, Joo EK, Roh KH, Seo MS, Han TH, Lee SY, Ryu PD, Jung JW, Seo KW, Kang SK, Kang KS.

Cell Transplant. 2011;20(7):1033-47. doi: 10.3727/096368910X545086.

PMID:
21176403
3.

The C57BL/6J Niemann-Pick C1 mouse model with decreased gene dosage has impaired glucose tolerance independent of body weight.

Jelinek D, Castillo JJ, Garver WS.

Gene. 2013 Sep 15;527(1):65-70. doi: 10.1016/j.gene.2013.05.080.

4.

The natural history of cerebellar degeneration of Niemann-Pick C mice monitored in vitro.

Marschalek N, Albert F, Meske V, Ohm TG.

Neuropathol Appl Neurobiol. 2014 Dec;40(7):933-45. doi: 10.1111/nan.12154.

PMID:
24889722
5.

Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann-Pick type C disease.

Alvarez AR, Klein A, Castro J, Cancino GI, Amigo J, Mosqueira M, Vargas LM, Yévenes LF, Bronfman FC, Zanlungo S.

FASEB J. 2008 Oct;22(10):3617-27. doi: 10.1096/fj.07-102715.

6.

Geranylgeranyl pyrophosphate is crucial for neuronal survival but has no special role in Purkinje cell degeneration in Niemann Pick type C1 disease.

Marschalek N, Albert F, Afshordel S, Meske V, Eckert GP, Ohm TG.

J Neurochem. 2015 Apr;133(1):153-61. doi: 10.1111/jnc.12959.

7.

Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

Repa JJ, Li H, Frank-Cannon TC, Valasek MA, Turley SD, Tansey MG, Dietschy JM.

J Neurosci. 2007 Dec 26;27(52):14470-80.

8.

Vitamin E dietary supplementation improves neurological symptoms and decreases c-Abl/p73 activation in Niemann-Pick C mice.

Marín T, Contreras P, Castro JF, Chamorro D, Balboa E, Bosch-Morató M, Muñoz FJ, Alvarez AR, Zanlungo S.

Nutrients. 2014 Jul 30;6(8):3000-17. doi: 10.3390/nu6083000.

9.
10.

Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.

Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP.

Hum Mol Genet. 2010 Mar 1;19(5):837-47. doi: 10.1093/hmg/ddp552.

11.

Miglustat improves purkinje cell survival and alters microglial phenotype in feline Niemann-Pick disease type C.

Stein VM, Crooks A, Ding W, Prociuk M, O'Donnell P, Bryan C, Sikora T, Dingemanse J, Vanier MT, Walkley SU, Vite CH.

J Neuropathol Exp Neurol. 2012 May;71(5):434-48. doi: 10.1097/NEN.0b013e31825414a6.

12.

A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.

Maue RA, Burgess RW, Wang B, Wooley CM, Seburn KL, Vanier MT, Rogers MA, Chang CC, Chang TY, Harris BT, Graber DJ, Penatti CA, Porter DM, Szwergold BS, Henderson LP, Totenhagen JW, Trouard TP, Borbon IA, Erickson RP.

Hum Mol Genet. 2012 Feb 15;21(4):730-50. doi: 10.1093/hmg/ddr505.

13.

Changes of calcium binding proteins, c-Fos and COX in hippocampal formation and cerebellum of Niemann-Pick, type C mouse.

Byun K, Kim D, Bayarsaikhan E, Oh J, Kim J, Kwak G, Jeong GB, Jo SM, Lee B.

J Chem Neuroanat. 2013 Sep;52:1-8. doi: 10.1016/j.jchemneu.2013.04.006.

PMID:
23660496
14.

Characterization of cognitive deficits in a transgenic mouse model of Alzheimer's disease and effects of donepezil and memantine.

Nagakura A, Shitaka Y, Yarimizu J, Matsuoka N.

Eur J Pharmacol. 2013 Mar 5;703(1-3):53-61. doi: 10.1016/j.ejphar.2012.12.023.

PMID:
23276665
15.

Cerebellar long-term depression is deficient in Niemann-Pick type C disease mice.

Sun CL, Su LD, Li Q, Wang XX, Shen Y.

Cerebellum. 2011 Mar;10(1):88-95. doi: 10.1007/s12311-010-0233-2.

PMID:
21086197
16.

Altered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brains.

Kodam A, Maulik M, Peake K, Amritraj A, Vetrivel KS, Thinakaran G, Vance JE, Kar S.

Glia. 2010 Aug 15;58(11):1267-81. doi: 10.1002/glia.21001.

17.

Adipose tissue-derived stem cells rescue Purkinje neurons and alleviate inflammatory responses in Niemann-Pick disease type C mice.

Bae JS, Carter JE, Jin HK.

Cell Tissue Res. 2010 May;340(2):357-69. doi: 10.1007/s00441-010-0942-3.

PMID:
20238127
18.

Alteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stress.

Vázquez MC, del Pozo T, Robledo FA, Carrasco G, Pavez L, Olivares F, González M, Zanlungo S.

PLoS One. 2011;6(12):e28777. doi: 10.1371/journal.pone.0028777.

19.

Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brain.

Kennedy BE, LeBlanc VG, Mailman TM, Fice D, Burton I, Karakach TK, Karten B.

PLoS One. 2013 Dec 18;8(12):e82685. doi: 10.1371/journal.pone.0082685.

20.

Polyphenol-rich black chokeberry (Aronia melanocarpa) extract regulates the expression of genes critical for intestinal cholesterol flux in Caco-2 cells.

Kim B, Park Y, Wegner CJ, Bolling BW, Lee J.

J Nutr Biochem. 2013 Sep;24(9):1564-70. doi: 10.1016/j.jnutbio.2013.01.005.

PMID:
23517916
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