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Results: 1 to 20 of 108

1.

Rescue of neurodegeneration in Niemann-Pick C mice by a prion-promoter-driven Npc1 cDNA transgene.

Loftus SK, Erickson RP, Walkley SU, Bryant MA, Incao A, Heidenreich RA, Pavan WJ.

Hum Mol Genet. 2002 Nov 15;11(24):3107-14.

PMID:
12417532
[PubMed - indexed for MEDLINE]
Free Article
2.

Studies on neuronal death in the mouse model of Niemann-Pick C disease.

Erickson RP, Bernard O.

J Neurosci Res. 2002 Jun 15;68(6):738-44.

PMID:
12111834
[PubMed - indexed for MEDLINE]
3.

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.

PMID:
18160655
[PubMed - indexed for MEDLINE]
Free Article
4.

Neurodegeneration in heterozygous Niemann-Pick type C1 (NPC1) mouse: implication of heterozygous NPC1 mutations being a risk for tauopathy.

Yu W, Ko M, Yanagisawa K, Michikawa M.

J Biol Chem. 2005 Jul 22;280(29):27296-302. Epub 2005 May 25.

PMID:
15919659
[PubMed - indexed for MEDLINE]
Free Article
5.

Decreased estradiol release from astrocytes contributes to the neurodegeneration in a mouse model of Niemann-Pick disease type C.

Chen G, Li HM, Chen YR, Gu XS, Duan S.

Glia. 2007 Nov 15;55(15):1509-18.

PMID:
17705200
[PubMed - indexed for MEDLINE]
6.

Sperm defects in mice lacking a functional Niemann-Pick C1 protein.

Fan J, Akabane H, Graham SN, Richardson LL, Zhu GZ.

Mol Reprod Dev. 2006 Oct;73(10):1284-91.

PMID:
16850391
[PubMed - indexed for MEDLINE]
7.

Regional and developmental expression of the Npc1 mRNA in the mouse brain.

Prasad A, Fischer WA, Maue RA, Henderson LP.

J Neurochem. 2000 Sep;75(3):1250-7.

PMID:
10936208
[PubMed - indexed for MEDLINE]
8.

Consequences of NPC1 and NPC2 loss of function in mammalian neurons.

Walkley SU, Suzuki K.

Biochim Biophys Acta. 2004 Oct 11;1685(1-3):48-62. Review.

PMID:
15465426
[PubMed - indexed for MEDLINE]
Free Article
9.

Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent.

Gondré-Lewis MC, McGlynn R, Walkley SU.

Curr Biol. 2003 Aug 5;13(15):1324-9.

PMID:
12906793
[PubMed - indexed for MEDLINE]
Free Article
10.

Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia.

Baudry M, Yao Y, Simmons D, Liu J, Bi X.

Exp Neurol. 2003 Dec;184(2):887-903.

PMID:
14769381
[PubMed - indexed for MEDLINE]
11.

A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain.

Reid PC, Sakashita N, Sugii S, Ohno-Iwashita Y, Shimada Y, Hickey WF, Chang TY.

J Lipid Res. 2004 Mar;45(3):582-91. Epub 2004 Jan 1.

PMID:
14703504
[PubMed - indexed for MEDLINE]
Free Article
12.

Characterization of liver disease and lipid metabolism in the Niemann-Pick C1 mouse.

Garver WS, Jelinek D, Oyarzo JN, Flynn J, Zuckerman M, Krishnan K, Chung BH, Heidenreich RA.

J Cell Biochem. 2007 May 15;101(2):498-516.

PMID:
17216601
[PubMed - indexed for MEDLINE]
13.

Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse.

Liu B, Turley SD, Burns DK, Miller AM, Repa JJ, Dietschy JM.

Proc Natl Acad Sci U S A. 2009 Feb 17;106(7):2377-82. doi: 10.1073/pnas.0810895106. Epub 2009 Jan 26.

PMID:
19171898
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse.

Liu Y, Wu YP, Wada R, Neufeld EB, Mullin KA, Howard AC, Pentchev PG, Vanier MT, Suzuki K, Proia RL.

Hum Mol Genet. 2000 Apr 12;9(7):1087-92.

PMID:
10767333
[PubMed - indexed for MEDLINE]
Free Article
15.

Genetic variations and treatments that affect the lifespan of the NPC1 mouse.

Liu B, Li H, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2008 Mar;49(3):663-9. Epub 2007 Dec 12.

PMID:
18077828
[PubMed - indexed for MEDLINE]
Free Article
16.

Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease.

Langmade SJ, Gale SE, Frolov A, Mohri I, Suzuki K, Mellon SH, Walkley SU, Covey DF, Schaffer JE, Ory DS.

Proc Natl Acad Sci U S A. 2006 Sep 12;103(37):13807-12. Epub 2006 Aug 29.

PMID:
16940355
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Adenovirus expressing an NPC1-GFP fusion gene corrects neuronal and nonneuronal defects associated with Niemann pick type C disease.

Paul CA, Reid PC, Boegle AK, Karten B, Zhang M, Jiang ZG, Franz D, Lin L, Chang TY, Vance JE, Blanchette-Mackie J, Maue RA.

J Neurosci Res. 2005 Sep 1;81(5):706-19.

PMID:
16015597
[PubMed - indexed for MEDLINE]
18.

Expression of Npc1 in glial cells corrects sterility in Npc1(-/-) mice.

Donohue C, Marion S, Erickson RP.

J Appl Genet. 2009;50(4):385-90. doi: 10.1007/BF03195698.

PMID:
19875890
[PubMed - indexed for MEDLINE]
19.

Neurodegeneration in the Niemann-Pick C mouse: glial involvement.

German DC, Liang CL, Song T, Yazdani U, Xie C, Dietschy JM.

Neuroscience. 2002;109(3):437-50.

PMID:
11823057
[PubMed - indexed for MEDLINE]
20.

Niemann-Pick C1 mice, a model of "juvenile Alzheimer's disease", with normal gene expression in neurons and fibrillary astrocytes show long term survival and delayed neurodegeneration.

Borbon I, Totenhagen J, Fiorenza MT, Canterini S, Ke W, Trouard T, Erickson RP.

J Alzheimers Dis. 2012;30(4):875-87. doi: 10.3233/JAD-2012-120199.

PMID:
22495346
[PubMed - indexed for MEDLINE]

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