NPC1
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Official Full Name
Niemann-Pick disease, type C1
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Overview
This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments. -
Synonyms
NPC1; Niemann-Pick disease, type C1; Niemann-Pick C1 protein; NPC; FLJ98532; Cdig2;
- Recombinant Proteins
- Human
- Zebrafish
- E.coli
- HEK293
- Mammalian Cell
- Wheat Germ
- Yeast
- GST
- His
- Myc
- His|Flag
- N/A
- N
| Species | Cat.# | Product name | Source (Host) | Tag | Protein Length | Price |
|---|---|---|---|---|---|---|
| Human | NPC1-1339H |
Recombinant Human NPC1 protein, His/FLAG-tagged
|
HEK293 | His/Flag | Arg372-Phe622 | |
| Human | NPC1-29133TH | Recombinant Human NPC1, GST-tagged | Wheat Germ | GST |
|
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| Human | NPC1-29132TH | Recombinant Human NPC1 | Wheat Germ | N/A | 100 amino acids |
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| Human | NPC1-1338H | Recombinant Human NPC1, His-tagged | E.coli | His | 23-266aa | |
| Human | NPC1-4432H | Recombinant Human NPC1 protein, His-Myc-tagged | Yeast | His/Myc | 23-261aa |
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| Human | NPC1-4725H | Recombinant Human NPC1 Protein (Gln23-Asp266), N-His tagged | E.coli | N-His | Gln23-Asp266 |
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| Zebrafish | NPC1-8054Z | Recombinant Zebrafish NPC1 | Mammalian Cell | His |
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- Involved Pathway
- Protein Function
- Interacting Protein
- NPC1 Related Articles
NPC1 involved in several pathways and played different roles in them. We selected most pathways NPC1 participated on our site, such as Lysosome, which may be useful for your reference. Also, other proteins which involved in the same pathway with NPC1 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.
| Pathway Name | Pathway Related Protein |
|---|---|
| Lysosome | PLA2G15;ASAH1B;CTSSB.1;CTSBB;CTSH;IDUA;AP1M3;MANBA;LITAF |
NPC1 has several biochemical functions, for example, cholesterol binding, hedgehog receptor activity, protein binding. Some of the functions are cooperated with other proteins, some of the functions could acted by NPC1 itself. We selected most functions NPC1 had, and list some proteins which have the same functions with NPC1. You can find most of the proteins on our site.
| Function | Related Protein |
|---|---|
| cholesterol binding | APOA2;APOEA;CETP;OSBP2;NPC1;ERLIN1;CYP11A1;ANXA6;STAR |
| hedgehog receptor activity | PTCH1;B9D1;PTCH2;PTCHD4;NPC1;DISP1;PTCHD3;PTCHD1 |
| protein binding | NDEL1;C1orf35;SLA2;LRCH3;MAPK13;ODF2;EIF3J;C16orf79;SPON1 |
| receptor activity | TIGIT;UNC13B;SELS;CD244;TNFRSF6B;NLGN4B;P2RY1;GFRA4A;P2RY11 |
| sterol transporter activity | NPC1;SCP2A;C20orf79;SCP2;OSBP |
| transmembrane signaling receptor activity | TNFRSF10D;GPR98;OR4D6;SORL1;CLDN4;CLDN3;IGSF6;CD79B;CLEC2D |
| virus receptor activity | HLA-DRB1;CD209A;CCR5;ANPEP;LAMP1;CD46;F11R;MOG;AXL |
NPC1 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with NPC1 here. Most of them are supplied by our site. Hope this information will be useful for your research of NPC1.
cholesterol; b3xyc5_sollc; cona_canen; TMEM173; Unc93b1
- Q&As
- Reviews
Q&As (6)
Ask a questionMutations in the NPC1 protein can lead to degenerative changes in the nervous system, enlargement of organs, intellectual disability, and other characteristics.
Studies have shown that NPC1 protein mutations may affect neuroinflammatory responses, but the specific mechanism needs to be further studied.
The NPC1 protein plays an important role in intracellular cholesterol transport and metabolism, helping cells regulate cholesterol levels.
NPC1 protein is mainly localized in the endoplasmic reticulum and Golgi apparatus, and is involved in cholesterol transport and metabolic regulation.
The full name of NPC1 protein is Niemann-Pick C1 protein.
Mutations in the NPC1 protein are associated with Niemann-Pick disease type C, a rare inherited metabolic disorder that affects nervous system and liver function.
Customer Reviews (3)
Write a reviewThe background is very clean and there is no immunogenicity.
The NPC1 is very productive and can meet a large number of my needs.
It is very stable and can be stored and used for a long time.
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