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Results: 1 to 20 of 227

1.

Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain.

Ohgami N, Ko DC, Thomas M, Scott MP, Chang CC, Chang TY.

Proc Natl Acad Sci U S A. 2004 Aug 24;101(34):12473-8. Epub 2004 Aug 16.

PMID:
15314240
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

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.

PMID:
20007703
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

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
[PubMed - indexed for MEDLINE]
Free Article
4.

The sterol-sensing domain of the Niemann-Pick C1 (NPC1) protein regulates trafficking of low density lipoprotein cholesterol.

Millard EE, Gale SE, Dudley N, Zhang J, Schaffer JE, Ory DS.

J Biol Chem. 2005 Aug 5;280(31):28581-90. Epub 2005 May 20.

PMID:
15908696
[PubMed - indexed for MEDLINE]
Free Article
5.

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.

PMID:
11349231
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Targeting of NPC1 to late endosomes involves multiple signals, including one residing within the putative sterol-sensing domain.

Scott C, Higgins ME, Davies JP, Ioannou YA.

J Biol Chem. 2004 Nov 12;279(46):48214-23. Epub 2004 Sep 3.

PMID:
15347664
[PubMed - indexed for MEDLINE]
Free Article
7.

Sterols and intracellular vesicular trafficking: lessons from the study of NPC1.

Strauss JF 3rd, Liu P, Christenson LK, Watari H.

Steroids. 2002 Nov;67(12):947-51.

PMID:
12398991
[PubMed - indexed for MEDLINE]
8.

Characterization of fluorescent sterol binding to purified human NPC1.

Liu R, Lu P, Chu JW, Sharom FJ.

J Biol Chem. 2009 Jan 16;284(3):1840-52. doi: 10.1074/jbc.M803741200. Epub 2008 Nov 24.

PMID:
19029290
[PubMed - indexed for MEDLINE]
Free Article
9.

Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease.

Zhang M, Dwyer NK, Love DC, Cooney A, Comly M, Neufeld E, Pentchev PG, Blanchette-Mackie EJ, Hanover JA.

Proc Natl Acad Sci U S A. 2001 Apr 10;98(8):4466-71.

PMID:
11296289
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Identification of 58 novel mutations in Niemann-Pick disease type C: correlation with biochemical phenotype and importance of PTC1-like domains in NPC1.

Park WD, O'Brien JF, Lundquist PA, Kraft DL, Vockley CW, Karnes PS, Patterson MC, Snow K.

Hum Mutat. 2003 Oct;22(4):313-25.

PMID:
12955717
[PubMed - indexed for MEDLINE]
11.

Differential trafficking of the Niemann-Pick C1 and 2 proteins highlights distinct roles in late endocytic lipid trafficking.

Zhang M, Sun M, Dwyer NK, Comly ME, Patel SC, Sundaram R, Hanover JA, Blanchette-Mackie EJ.

Acta Paediatr Suppl. 2003 Dec;92(443):63-73; discussion 45.

PMID:
14989468
[PubMed - indexed for MEDLINE]
12.

Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function.

Frolov A, Srivastava K, Daphna-Iken D, Traub LM, Schaffer JE, Ory DS.

J Biol Chem. 2001 Dec 7;276(49):46414-21. Epub 2001 Sep 24.

PMID:
11571306
[PubMed - indexed for MEDLINE]
Free Article
13.

Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein.

Watari H, Blanchette-Mackie EJ, Dwyer NK, Watari M, Neufeld EB, Patel S, Pentchev PG, Strauss JF 3rd.

J Biol Chem. 1999 Jul 30;274(31):21861-6.

PMID:
10419504
[PubMed - indexed for MEDLINE]
Free Article
14.

Cholesterol depletion facilitates ubiquitylation of NPC1 and its association with SKD1/Vps4.

Ohsaki Y, Sugimoto Y, Suzuki M, Hosokawa H, Yoshimori T, Davies JP, Ioannou YA, Vanier MT, Ohno K, Ninomiya H.

J Cell Sci. 2006 Jul 1;119(Pt 13):2643-53. Epub 2006 Jun 6.

PMID:
16757520
[PubMed - indexed for MEDLINE]
Free Article
15.

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
[PubMed - indexed for MEDLINE]
16.

The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system.

Garver WS, Heidenreich RA.

Curr Mol Med. 2002 Aug;2(5):485-505. Review.

PMID:
12125814
[PubMed - indexed for MEDLINE]
17.

Transport of plasma membrane-derived cholesterol and the function of Niemann-Pick C1 Protein.

Wiegand V, Chang TY, Strauss JF 3rd, Fahrenholz F, Gimpl G.

FASEB J. 2003 Apr;17(6):782-4. Epub 2003 Feb 19.

PMID:
12594172
[PubMed - indexed for MEDLINE]
Free Article
18.

Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease.

Blom TS, Linder MD, Snow K, Pihko H, Hess MW, Jokitalo E, Veckman V, Syvänen AC, Ikonen E.

Hum Mol Genet. 2003 Feb 1;12(3):257-72.

PMID:
12554680
[PubMed - indexed for MEDLINE]
Free Article
19.

Niemann-Pick type C disease: importance of N-glycosylation sites for function and cellular location of the NPC2 protein.

Chikh K, Vey S, Simonot C, Vanier MT, Millat G.

Mol Genet Metab. 2004 Nov;83(3):220-30.

PMID:
15542393
[PubMed - indexed for MEDLINE]
20.

Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells.

Karten B, Peake KB, Vance JE.

Biochim Biophys Acta. 2009 Jul;1791(7):659-70. doi: 10.1016/j.bbalip.2009.01.025. Epub 2009 Feb 11. Review.

PMID:
19416638
[PubMed - indexed for MEDLINE]
Free Article

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