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Human Niemann Pick C1 ELISA Kit is a Sandwich (quantitative) ELISA kit for the measurement of Human Niemann Pick C1 in Human in Cell culture supernatant, Plasma, Serum samples.

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Images

Sandwich ELISA - Human Niemann Pick C1 ELISA Kit (AB313912), expandable thumbnail

Key facts

Detection method
Colorimetric
Sample types
Cell culture supernatant, Plasma, Serum
Assay type
Sandwich (quantitative)
Reactive species
Human
Range
0.12 - 30 ng/mL
Sensitivity
= 0.12 ng/mL

Reactivity data

Application
sELISA
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

What's included?

1 x 96 Tests
Components
20X Wash Buffer
1 x 25 mL
5X Assay Diluent (Item E2)
1 x 15 mL
600X HRP-Streptavidin
1 x 200 µL
Biotinylated Human Niemann Pick C1 Detection Antibody
2 x 1 Vial
Human Niemann Pick C1 Antibody-coated ELISA Plate
1 x 1 Unit
Lyophilized Human Niemann Pick C1 Protein Standard
2 x 1 Vial
Stop Solution; 0.2M Sulfuric Acid
1 x 8 mL
TMB One-Step Substrate Reagent
1 x 12 mL

Recommended products

Human Niemann Pick C1 ELISA Kit is a Sandwich (quantitative) ELISA kit for the measurement of Human Niemann Pick C1 in Human in Cell culture supernatant, Plasma, Serum samples.

Key facts

Detection method
Colorimetric
Sample types
Cell culture supernatant, Plasma, Serum
Assay type
Sandwich (quantitative)
Reactive species
Human
Range
0.12 - 30 ng/mL
Assay Platform
Pre-coated microplate (12 x 8 well strips)
Sensitivity
= 0.12 ng/mL

Recovery

Sample specific recovery

Sample type
Serum
Average %
= 97.08
Range
70 - 122 %
Sample type
Plasma
Average %
= 96.49
Range
77 - 113 %
Sample type
Cell culture media
Average %
= 114.5
Range
96 - 124 %

Storage

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Storage information
-20°C

Notes

Human Niemann Pick C1 (NPC intracellular cholesterol transporter 1) ELISA kit is an in vitro enzyme-linked immunosorbent assay for the quantitative measurement of human Niemann Pick C1 in serum, plasma and cell culture supernatants. This assay employs an antibody specific for human Niemann Pick C1 coated on a 96-well plate.

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

  • Sandwich ELISA - Human Niemann Pick C1 ELISA Kit (ab313912), expandable thumbnail

    Sandwich ELISA - Human Niemann Pick C1 ELISA Kit (ab313912)

    Example of standard curve using ab313912. This standard curve is for demonstrative purposes only. A standard curve must be run with each assay.

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can:

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