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Items: 1 to 20 of 101

1.

Binding and intracellular transport of 25-hydroxycholesterol by Niemann-Pick C2 protein.

Petersen D, Reinholdt P, Szomek M, Hansen SK, Poongavanam V, Dupont A, Heegaard CW, Krishnan K, Fujiwara H, Covey DF, Ory DS, Kongsted J, Wüstner D.

Biochim Biophys Acta Biomembr. 2019 Sep 12:183063. doi: 10.1016/j.bbamem.2019.183063. [Epub ahead of print]

PMID:
31521631
2.

Niemann-Pick C2 protein regulates sterol transport between plasma membrane and late endosomes in human fibroblasts.

Berzina Z, Solanko LM, Mehadi AS, Jensen MLV, Lund FW, Modzel M, Szomek M, Solanko KA, Dupont A, Nielsen GK, Heegaard CW, Ejsing CS, Wüstner D.

Chem Phys Lipids. 2018 Jul;213:48-61. doi: 10.1016/j.chemphyslip.2018.03.006. Epub 2018 Mar 23.

PMID:
29580834
3.

NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols.

Liou HL, Dixit SS, Xu S, Tint GS, Stock AM, Lobel P.

J Biol Chem. 2006 Dec 1;281(48):36710-23. Epub 2006 Oct 2.

4.

Regulation of sterol transport between membranes and NPC2.

Xu Z, Farver W, Kodukula S, Storch J.

Biochemistry. 2008 Oct 21;47(42):11134-43. doi: 10.1021/bi801328u. Epub 2008 Sep 30.

5.

Live-cell imaging of new polyene sterols for improved analysis of intracellular cholesterol transport.

Modzel M, Solanko KA, Szomek M, Hansen SK, Dupont A, Nåbo LJ, Kongsted J, Wüstner D.

J Microsc. 2018 Jul;271(1):36-48. doi: 10.1111/jmi.12691. Epub 2018 Mar 8.

PMID:
29516493
6.

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.

7.

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
8.

Structural basis of sterol binding by NPC2, a lysosomal protein deficient in Niemann-Pick type C2 disease.

Xu S, Benoff B, Liou HL, Lobel P, Stock AM.

J Biol Chem. 2007 Aug 10;282(32):23525-31. Epub 2007 Jun 14.

9.

Sterol transfer between cyclodextrin and membranes: similar but not identical mechanism to NPC2-mediated cholesterol transfer.

McCauliff LA, Xu Z, Storch J.

Biochemistry. 2011 Aug 30;50(34):7341-7349. doi: 10.1021/bi200574f. Epub 2011 Aug 4.

10.

Mannose 6-phosphate receptors, Niemann-Pick C2 protein, and lysosomal cholesterol accumulation.

Willenborg M, Schmidt CK, Braun P, Landgrebe J, von Figura K, Saftig P, Eskelinen EL.

J Lipid Res. 2005 Dec;46(12):2559-69. Epub 2005 Sep 21.

11.

Multiple Surface Regions on the Niemann-Pick C2 Protein Facilitate Intracellular Cholesterol Transport.

McCauliff LA, Xu Z, Li R, Kodukula S, Ko DC, Scott MP, Kahn PC, Storch J.

J Biol Chem. 2015 Nov 6;290(45):27321-31. doi: 10.1074/jbc.M115.667469. Epub 2015 Aug 20.

12.

NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols.

Frolov A, Zielinski SE, Crowley JR, Dudley-Rucker N, Schaffer JE, Ory DS.

J Biol Chem. 2003 Jul 11;278(28):25517-25. Epub 2003 Apr 28.

13.

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
14.

Mechanism of cholesterol transfer from the Niemann-Pick type C2 protein to model membranes supports a role in lysosomal cholesterol transport.

Cheruku SR, Xu Z, Dutia R, Lobel P, Storch J.

J Biol Chem. 2006 Oct 20;281(42):31594-604. Epub 2006 Apr 10.

15.
16.

Niemann-Pick type C disease: a QM/MM study of conformational changes in cholesterol in the NPC1(NTD) and NPC2 binding pockets.

Elghobashi-Meinhardt N.

Biochemistry. 2014 Oct 21;53(41):6603-14. doi: 10.1021/bi500548f. Epub 2014 Oct 10.

PMID:
25251378
17.

Concerted regulation of npc2 binding to endosomal/lysosomal membranes by bis(monoacylglycero)phosphate and sphingomyelin.

Enkavi G, Mikkolainen H, Güngör B, Ikonen E, Vattulainen I.

PLoS Comput Biol. 2017 Oct 30;13(10):e1005831. doi: 10.1371/journal.pcbi.1005831. eCollection 2017 Oct.

18.

Niemann-Pick C2 (NPC2) and intracellular cholesterol trafficking.

Storch J, Xu Z.

Biochim Biophys Acta. 2009 Jul;1791(7):671-8. doi: 10.1016/j.bbalip.2009.02.001. Epub 2009 Feb 13. Review.

19.

Protein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 disease.

Nielsen GK, Dagnaes-Hansen F, Holm IE, Meaney S, Symula D, Andersen NT, Heegaard CW.

PLoS One. 2011;6(11):e27287. doi: 10.1371/journal.pone.0027287. Epub 2011 Nov 3.

20.

Computational Analysis of Sterol Ligand Specificity of the Niemann Pick C2 Protein.

Poongavanam V, Kongsted J, Wüstner D.

Biochemistry. 2016 Sep 13;55(36):5165-79. doi: 10.1021/acs.biochem.6b00217. Epub 2016 Aug 25.

PMID:
27533706

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