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Items: 1 to 20 of 85


TNF-{alpha} plays a role in hepatocyte apoptosis in Niemann-Pick type C liver disease.

Rimkunas VM, Graham MJ, Crooke RM, Liscum L.

J Lipid Res. 2009 Feb;50(2):327-33. doi: 10.1194/jlr.M800415-JLR200. Epub 2008 Sep 24.


Evaluation of an anti-tumor necrosis factor therapeutic in a mouse model of Niemann-Pick C liver disease.

Vincent M, Sayre NL, Graham MJ, Crooke RM, Shealy DJ, Liscum L.

PLoS One. 2010 Sep 23;5(9):e12941. doi: 10.1371/journal.pone.0012941.


Recovery from liver disease in a Niemann-Pick type C mouse model.

Sayre NL, Rimkunas VM, Graham MJ, Crooke RM, Liscum L.

J Lipid Res. 2010 Aug;51(8):2372-83. doi: 10.1194/jlr.M007211. Epub 2010 Apr 24.


Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease.

Beltroy EP, Richardson JA, Horton JD, Turley SD, Dietschy JM.

Hepatology. 2005 Oct;42(4):886-93.


Niemann-Pick Type C1 deficiency in microglia does not cause neuron death in vitro.

Peake KB, Campenot RB, Vance DE, Vance JE.

Biochim Biophys Acta. 2011 Sep;1812(9):1121-9. doi: 10.1016/j.bbadis.2011.06.003. Epub 2011 Jun 17.


Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease.

Beltroy EP, Liu B, Dietschy JM, Turley SD.

J Lipid Res. 2007 Apr;48(4):869-81. Epub 2007 Jan 14.


Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.

Wu YP, Mizukami H, Matsuda J, Saito Y, Proia RL, Suzuki K.

Mol Genet Metab. 2005 Jan;84(1):9-17.


Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1.

Sun X, Marks DL, Park WD, Wheatley CL, Puri V, O'Brien JF, Kraft DL, Lundquist PA, Patterson MC, Pagano RE, Snow K.

Am J Hum Genet. 2001 Jun;68(6):1361-72. Epub 2001 May 9.


In vivo antisense oligonucleotide reduction of NPC1 expression as a novel mouse model for Niemann Pick type C- associated liver disease.

Rimkunas VM, Graham MJ, Crooke RM, Liscum L.

Hepatology. 2008 May;47(5):1504-12. doi: 10.1002/hep.22327.


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.


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. Epub 2011 Dec 22.


ABCA1-dependent mobilization of lysosomal cholesterol requires functional Niemann-Pick C2 but not Niemann-Pick C1 protein.

Boadu E, Nelson RC, Francis GA.

Biochim Biophys Acta. 2012 Mar;1821(3):396-404. doi: 10.1016/j.bbalip.2011.11.013. Epub 2011 Dec 10.


Defective insulin receptor activation and altered lipid rafts in Niemann-Pick type C disease hepatocytes.

Vainio S, Bykov I, Hermansson M, Jokitalo E, Somerharju P, Ikonen E.

Biochem J. 2005 Nov 1;391(Pt 3):465-72.


Differential regulation of ATP binding cassette protein A1 expression and ApoA-I lipidation by Niemann-Pick type C1 in murine hepatocytes and macrophages.

Wang MD, Franklin V, Sundaram M, Kiss RS, Ho K, Gallant M, Marcel YL.

J Biol Chem. 2007 Aug 3;282(31):22525-33. Epub 2007 Jun 5.


Niemann-Pick C1 functions independently of Niemann-Pick C2 in the initial stage of retrograde transport of membrane-impermeable lysosomal cargo.

Goldman SD, Krise JP.

J Biol Chem. 2010 Feb 12;285(7):4983-94. doi: 10.1074/jbc.M109.037622. Epub 2009 Dec 10.


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. Epub 2008 Jun 30.


Lysosomal vitamin E accumulation in Niemann-Pick type C disease.

Yévenes LF, Klein A, Castro JF, Marín T, Leal N, Leighton F, Alvarez AR, Zanlungo S.

Biochim Biophys Acta. 2012 Feb;1822(2):150-60. doi: 10.1016/j.bbadis.2011.11.009. Epub 2011 Nov 15.


Critical role for glycosphingolipids in Niemann-Pick disease type C.

Zervas M, Somers KL, Thrall MA, Walkley SU.

Curr Biol. 2001 Aug 21;11(16):1283-7.


Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease.

Li H, Repa JJ, Valasek MA, Beltroy EP, Turley SD, German DC, Dietschy JM.

J Neuropathol Exp Neurol. 2005 Apr;64(4):323-33.


Increased copper levels in in vitro and in vivo models of Niemann-Pick C disease.

Vázquez MC, Martínez P, Alvarez AR, González M, Zanlungo S.

Biometals. 2012 Aug;25(4):777-86. doi: 10.1007/s10534-012-9546-6. Epub 2012 Apr 17.

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