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NPC / NPC1

Niemann-Pick disease, type C1

Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.

Gene Name:	Niemann-Pick disease, type C1
Synonyms:	NPC1, NPC, Niemann-Pick C1 protein, Niemann-Pick disease, type C1
Target Sequences:	NM_000271

Publications (18)

Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS. The Journal of biological chemistry. 2000 275:38445-51. (ICC, WB) [PubMed:10964915]

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. The Journal of biological chemistry. 2001 276:46414-21. (ICC) [PubMed:11571306]

Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. Infante RE, Abi-Mosleh L, Radhakrishnan A, Dale JD, Brown MS, Goldstein JL. The Journal of biological chemistry. 2008 283:1052-63. (WB; Human) [PubMed:17989073]

Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding. Gelsthorpe ME, Baumann N, Millard E, Gale SE, Langmade SJ, Schaffer JE, Ory DS. The Journal of biological chemistry. 2008 283:8229-36. (IP) [PubMed:18216017] [PMC:PMC2276376]

Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the golden Syrian hamster. Burke KT, Colvin PL, Myatt L, Graf GA, Schroeder F, Woollett LA. Journal of lipid research. 2009 50:1146-55. [PubMed:19122238] [PMC:PMC2681396]

NPC1 repression contributes to lipid accumulation in human macrophages exposed to environmental aryl hydrocarbons. Podechard N, Le Ferrec E, Rebillard A, Fardel O, Lecureur V. Cardiovascular research. 2009 82:361-70. [PubMed:19131362]

Cholesterol sensor ORP1L contacts the ER protein VAP to control Rab7-RILP-p150 Glued and late endosome positioning. Rocha N, Kuijl C, van der Kant R, Janssen L, Houben D, Janssen H, Zwart W, Neefjes J. The Journal of cell biology. 2009 185:1209-25. [PubMed:19564404] [PMC:PMC2712958]

Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells. Abi-Mosleh L, Infante RE, Radhakrishnan A, Goldstein JL, Brown MS. Proceedings of the National Academy of Sciences of the United States of America. 2009 106:19316-21. [PubMed:19884502] [PMC:PMC2780767]

MLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 protein. Charman M, Kennedy BE, Osborne N, Karten B. Journal of lipid research. 2010 51:1023-34. (WB; Mouse) [PubMed:19965586] [PMC:PMC2853429]

FTY720 stimulates 27-hydroxycholesterol production and confers atheroprotective effects in human primary macrophages. Blom T, Bck N, Mutka AL, Bittman R, Li Z, de Lera A, Kovanen PT, Diczfalusy U, Ikonen E. Circulation research. 2010 106:720-9. [PubMed:20056921] [PMC:PMC2871287]

Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder. Lopez ME, Klein AD, Dimbil UJ, Scott MP. The Journal of neuroscience : the official journal of the Society for Neuroscience. 2011 31:4367-78. [PubMed:21430138] [PMC:PMC3071647]

Imaging the impact of Nox4 in cycling hypoxia-mediated U87 glioblastoma invasion and infiltration. Hsieh CH, Chang HT, Shen WC, Shyu WC, Liu RS. Molecular imaging and biology : MIB : the official publication of the Academy of Molecular Imaging. 2012 14:489-99. (IHC; Mouse) [PubMed:21870211]

A novel role for -tocopherol transfer protein (-TTP) in protecting against chloroquine toxicity. Shichiri M, Kono N, Shimanaka Y, Tanito M, Rotzoll DE, Yoshida Y, Hagihara Y, Tamai H, Arai H. The Journal of biological chemistry. 2012 287:2926-34. (WB; Rat) [PubMed:22147702] [PMC:PMC3268449]

Subversion of NPC1 pathway of cholesterol transport by Anaplasma phagocytophilum. Xiong Q, Rikihisa Y. Cellular microbiology. 2012 14:560-76. (ICC) [PubMed:22212234] [PMC:PMC3604884]

Loss of Niemann Pick type C proteins 1 and 2 greatly enhances HIV infectivity and is associated with accumulation of HIV Gag and cholesterol in late endosomes/lysosomes. Coleman EM, Walker TN, Hildreth JE. Virology journal. 2012 9:31. [PubMed:22273177] [PMC:PMC3299633]

Tracking sphingosine metabolism and transport in sphingolipidoses: NPC1 deficiency as a test case. Blom T, Li Z, Bittman R, Somerharju P, Ikonen E. Traffic (Copenhagen, Denmark). 2012 13:1234-43. (WB; Human) [PubMed:22607065]

An essential role of Hrs/Vps27 in endosomal cholesterol trafficking. Du X, Kazim AS, Brown AJ, Yang H. Cell reports. 2012 1:29-35. (WB; Human) [PubMed:22832105]

Induction of Cell-Cell Fusion by Ebola Virus Glycoprotein: Low pH Is Not a Trigger. Markosyan RM, Miao C, Zheng YM, Melikyan GB, Liu SL, Cohen FS. PLoS pathogens. 2016 12:e1005373. (WB; Human) [Full Text Article] [PubMed:26730950] [PMC:PMC4711667] Related Antibodies: LS-C285835.

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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).

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