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AB276725

Recombinant Human Niemann Pick C1 protein (His tag)

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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 cells, with >95%, < 1 EU/µg endotoxin level, suitable for SDS-PAGE.

View Alternative Names

NPC intracellular cholesterol transporter 1, Niemann-Pick C1 protein, NPC1

1 Images
SDS-PAGE - Recombinant Human Niemann Pick C1 protein (His tag) (AB276725)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human Niemann Pick C1 protein (His tag) (AB276725)

SDS-PAGE analysis of ab276725

Key facts

Purity

>95% SDS-PAGE

Endotoxin level

< 1 EU/µg

Expression system

HEK 293 cells

Tags

His tag N-Terminus DDDDK tag N-Terminus

Applications

SDS-PAGE

applications

Biologically active

No

Accession

O15118

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 7.4 Constituents: PBS, 5% Trehalose, 5% Mannitol, 0.01% Tween 80

storage-buffer

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Complementary Products

Reagents, Controls & Accessories

AB325994

Prestained Protein Ladder - Mid-range molecular weight (11-180 kDa, 4-color)

SDS-PAGE - Prestained Protein Ladder - Mid-range molecular weight (11-180 kDa, 4-color) (AB325994)

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Sample Prep & Detection Kits

AB102918

PE / R-Phycoerythrin Conjugation Kit - Lightning-Link®

Flow Cytometry - PE / R-Phycoerythrin Conjugation Kit - Lightning-Link® (AB102918)

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Primary Antibodies

AB134113

Anti-Niemann Pick C1 antibody [EPR5209]

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Immunocytochemistry/ Immunofluorescence - Anti-Niemann Pick C1 antibody [EPR5209] (AB134113)

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Secondary Antibodies

AB125912

Rabbit monoclonal [H26-10] Anti-Human IgG1 H&L

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ELISA - Rabbit monoclonal [H26-10] Anti-Human IgG1 H&L (AB125912)

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Proteins & Peptides

AB151362

Recombinant Human EPCR/CD201 protein

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"RVTTNPVDLWSAPSSQARLEKEYFDQHFGPFFRTEQLIIRAPLTDKHIYQPYPSGADVPFGPPLDIQILHQVLDLQIAIENITASYDNETVTLQDICLAPLSPYNTNCTILSVLNYFQNSHSVLDHKKGDDFFVYADYHTHFLYCVRAPASLNDTSLLHDPCLGTFGGPVFPWLVLGGYDDQNYNNATALVITFPVNNYYNDTEKLQRAQAWEKEFINFVKNYKNPNLTISFTAERSIEDELNRESDSDVF","proteinLength":"Fragment","predictedMolecularWeight":"32 kDa","actualMolecularWeight":null,"aminoAcidEnd":622,"aminoAcidStart":372,"nature":"Recombinant","expressionSystem":"HEK 293 cells","accessionNumber":"O15118","tags":[{"tag":"His","terminus":"N-Terminus"},{"tag":"DDDDK","terminus":"N-Terminus"}]}]
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Properties and storage information

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
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

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.

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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Specifications

Form

Lyophilized

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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).. (Microbial infection) Acts as an endosomal entry receptor for ebolavirus.

Sequence similarities

Belongs to the patched family.

Post-translational modifications

N-glycosylated.

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Product protocols

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Target data

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.
See full target information NPC intracellular cholesterol transporter 1
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