Recombinant Human Niemann Pick C1 protein (His tag) is a Human Fragment protein, in the 372 to 622 aa range, expressed in HEK 293, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE.
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Key facts
- Purity
- >95% SDS-PAGE
- Endotoxin level
- < 1 EU/µg
- Expression system
- HEK 293 cells
- Tags
- His tag N-Terminus
- Applications
- SDS-PAGE
- Biologically active
- No
Amino acid sequence
R V T T N P V D L W S A P S S Q A R L E K E Y F D Q H F G P F F R T E Q L I I R A P L T D K H I Y Q P Y P S G A D V P F G P P L D I Q I L H Q V L D L Q I A I E N I T A S Y D N E T V T L Q D I C L A P L S P Y N T N C T I L S V L N Y F Q N S H S V L D H K K G D D F F V Y A D Y H T H F L Y C V R A P A S L N D T S L L H D P C L G T F G G P V F P W L V L G G Y D D Q N Y N N A T A L V I T F P V N N Y Y N D T E K L Q R A Q A W E K E F I N F V K N Y K N P N L T I S F T A E R S I E D E L N R E S D S D V F
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
Target data
Function
Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment (PubMed:10821832, PubMed:12554680, PubMed:18772377, PubMed:27238017, PubMed:9211849, PubMed:9927649). Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1 (PubMed:18772377, PubMed:19563754, PubMed:27238017, PubMed:27378690, PubMed:28784760, PubMed:9211849, PubMed:9927649). Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket (PubMed:19563754). 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 (Probable). Inhibits cholesterol-mediated mTORC1 activation throught its interaction with SLC38A9 (PubMed:28336668). (Microbial infection) Acts as an endosomal entry receptor for ebolavirus.
Alternative names
NPC intracellular cholesterol transporter 1, Niemann-Pick C1 protein, NPC1
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Recombinant Human Niemann Pick C1 protein (His tag) is a Human Fragment protein, in the 372 to 622 aa range, expressed in HEK 293, with >95% purity, < 1 EU/µg endotoxin level and suitable for SDS-PAGE.
Key facts
- Purity
- >95% SDS-PAGE
- Endotoxin level
- < 1 EU/µg
- Expression system
- HEK 293 cells
- Tags
- His tag N-Terminus
- Applications
- SDS-PAGE
- Biologically active
- No
- Accession
- O15118-1
- Animal free
- No
- Species
- Human
- Concentration
- Storage buffer
pH: 7.4
Constituents: PBS, 5% Mannitol, 5% Trehalose, 0.01% Tween 80
Sequence info
Amino acid sequence
- R V T T N P V D L W S A P S S Q A R L E K E Y F D Q H F G P F F R T E Q L I I R A P L T D K H I Y Q P Y P S G A D V P F G P P L D I Q I L H Q V L D L Q I A I E N I T A S Y D N E T V T L Q D I C L A P L S P Y N T N C T I L S V L N Y F Q N S H S V L D H K K G D D F F V Y A D Y H T H F L Y C V R A P A S L N D T S L L H D P C L G T F G G P V F P W L V L G G Y D D Q N Y N N A T A L V I T F P V N N Y Y N D T E K L Q R A Q A W E K E F I N F V K N Y K N P N L T I S F T A E R S I E D E L N R E S D S D V F
- Accession
- O15118
- Protein length
- Fragment
- Predicted molecular weight
- 32 kDa
- Amino acids
- 372 to 622
- Nature
- Recombinant
- Tags
- His tag N-Terminus
Specifications
- Form
- Lyophilized
General info
- Function
Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment (PubMed:10821832, PubMed:12554680, PubMed:18772377, PubMed:27238017, PubMed:9211849, PubMed:9927649). Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1 (PubMed:18772377, PubMed:19563754, PubMed:27238017, PubMed:27378690, PubMed:28784760, PubMed:9211849, PubMed:9927649). Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket (PubMed:19563754). 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 (Probable). Inhibits cholesterol-mediated mTORC1 activation throught its interaction with SLC38A9 (PubMed:28336668).
- Sequence similarities
Belongs to the patched family.
- Post-translational modifications
N-glycosylated.
- Subcellular localisation
- Late endosome membrane, Lysosome membrane
Storage
- Shipped at conditions
- Ambient - Can Ship with Ice
- Appropriate short-term storage conditions
- -20°C
- Appropriate long-term storage conditions
- -20°C
- Aliquoting information
- Upon delivery aliquot
- Storage information
- Avoid freeze / thaw cycle
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
Niemann Pick C1 also known as NPC1 or C1 protein is an integral membrane protein involved in cholesterol and lipid transport within cells. It is located in late endosomes and lysosomes and plays a role in intracellular cholesterol traffic. The NPC1 protein has a molecular weight of approximately 170 kDa. It is expressed in various tissues with high expression levels in the liver brain and spleen.
- Biological function summary
This protein acts in the movement of lipids and cholesterol through cellular compartments. NPC1 functions as part of a protein complex that includes NPC2 which works to mobilize cholesterol from lysosomes to other areas in the cell where it is further processed or stored. This transport mechanism is necessary to maintain cellular lipid homeostasis and proper cellular functions.
- Pathways
NPC1 integrates into the cholesterol trafficking pathway and plays an essential part in intracellular lipid regulation. It interacts with proteins involved in cholesterol metabolism such as sterol regulatory element-binding proteins (SREBPs). These interactions ensure that cholesterol distribution within the cell remains balanced and adjusts to metabolic needs. The proper function of NPC1 in these pathways is necessary for maintaining cell health and homeostasis.
- Associated diseases and disorders
NPC1 mutations are directly linked to Niemann-Pick disease type C a severe lipid storage disorder. This condition is characterized by the accumulation of cholesterol and other lipids in lysosomes leading to neurological and hepatic dysfunction. NPC1's role in this disease involves disrupted cholesterol trafficking which is critical for cellular lipid homeostasis. Additionally NPC2 protein is also affected in this disorder as both work together in the cholesterol transport process.
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1 product image
SDS-PAGE - Recombinant Human Niemann Pick C1 protein (His tag) (ab276725)
SDS-PAGE analysis of ab276725
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