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Items: 1 to 20 of 111

1.

Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.

Aqul A, Liu B, Ramirez CM, Pieper AA, Estill SJ, Burns DK, Liu B, Repa JJ, Turley SD, Dietschy JM.

J Neurosci. 2011 Jun 22;31(25):9404-13. doi: 10.1523/JNEUROSCI.1317-11.2011.

2.

Normalization of cholesterol homeostasis by 2-hydroxypropyl-β-cyclodextrin in neurons and glia from Niemann-Pick C1 (NPC1)-deficient mice.

Peake KB, Vance JE.

J Biol Chem. 2012 Mar 16;287(12):9290-8. doi: 10.1074/jbc.M111.326405. Epub 2012 Jan 25.

3.

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.

4.

Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.

Taylor AM, Liu B, Mari Y, Liu B, Repa JJ.

J Lipid Res. 2012 Nov;53(11):2331-42. doi: 10.1194/jlr.M028241. Epub 2012 Aug 14.

5.

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.

PMID:
22179027
6.

Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.

Ramirez CM, Liu B, Aqul A, Taylor AM, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2011 Apr;52(4):688-98. doi: 10.1194/jlr.M013789. Epub 2011 Feb 2.

7.

Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.

Zhou S, Davidson C, McGlynn R, Stephney G, Dobrenis K, Vanier MT, Walkley SU.

Am J Pathol. 2011 Aug;179(2):890-902. doi: 10.1016/j.ajpath.2011.04.017. Epub 2011 Jun 2.

8.

Sterol-modulated glycolipid sorting occurs in niemann-pick C1 late endosomes.

Zhang M, Dwyer NK, Neufeld EB, Love DC, Cooney A, Comly M, Patel S, Watari H, Strauss JF 3rd, Pentchev PG, Hanover JA, Blanchette-Mackie EJ.

J Biol Chem. 2001 Feb 2;276(5):3417-25. Epub 2000 Oct 13.

9.

Function of the Niemann-Pick type C proteins and their bypass by cyclodextrin.

Vance JE, Peake KB.

Curr Opin Lipidol. 2011 Jun;22(3):204-9. doi: 10.1097/MOL.0b013e3283453e69. Review.

PMID:
21412152
10.

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
11.

The Niemann-Pick C1 gene is downregulated by feedback inhibition of the SREBP pathway in human fibroblasts.

Garver WS, Jelinek D, Francis GA, Murphy BD.

J Lipid Res. 2008 May;49(5):1090-102. doi: 10.1194/jlr.M700555-JLR200. Epub 2008 Feb 13.

12.

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.

13.

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid.

Liu B, Ramirez CM, Miller AM, Repa JJ, Turley SD, Dietschy JM.

J Lipid Res. 2010 May;51(5):933-44. doi: 10.1194/jlr.M000257. Epub 2009 Nov 18.

14.

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.

15.

The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo.

Neufeld EB, Wastney M, Patel S, Suresh S, Cooney AM, Dwyer NK, Roff CF, Ohno K, Morris JA, Carstea ED, Incardona JP, Strauss JF 3rd, Vanier MT, Patterson MC, Brady RO, Pentchev PG, Blanchette-Mackie EJ.

J Biol Chem. 1999 Apr 2;274(14):9627-35.

17.

The transport of low density lipoprotein-derived cholesterol to the plasma membrane is defective in NPC1 cells.

Wojtanik KM, Liscum L.

J Biol Chem. 2003 Apr 25;278(17):14850-6. Epub 2003 Feb 18.

18.

Niemann-Pick C1 is a late endosome-resident protein that transiently associates with lysosomes and the trans-Golgi network.

Higgins ME, Davies JP, Chen FW, Ioannou YA.

Mol Genet Metab. 1999 Sep;68(1):1-13.

PMID:
10479477
19.

Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.

Ramirez CM, Liu B, Taylor AM, Repa JJ, Burns DK, Weinberg AG, Turley SD, Dietschy JM.

Pediatr Res. 2010 Oct;68(4):309-15. doi: 10.1203/00006450-201011001-00604.

20.

Cholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cells.

Ganley IG, Pfeffer SR.

J Biol Chem. 2006 Jun 30;281(26):17890-9. Epub 2006 Apr 26.

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