AB106534

Anti-Niemann Pick C1 antibody

Name: Anti-Niemann Pick C1 antibody (ab106534) | Abcam
Brand: Abcam Limited
SKU: AB106534
Price: 545 USD
Availability: InStock

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(3 Publications)

Rabbit Polyclonal Niemann Pick C1 antibody. Suitable for WB, IHC-P, ICC/IF and reacts with Human, Mouse samples. Cited in 3 publications. Immunogen corresponding to Synthetic Peptide within Human NPC intracellular cholesterol transporter 1.

View Alternative Names

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

2 Images

Supplier Data

Western blot - Anti-Niemann Pick C1 antibody (AB106534)

All lanes:

Western blot - Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/mL

All lanes:

HepG2 (human liver hepatocellular carcinoma cell line) cell lysate

Predicted band size: 142 kDa

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Western blot - Anti-Niemann Pick C1 antibody (AB106534)

All lanes:

Western blot - Anti-Niemann Pick C1 antibody (ab106534) at 1 µg/mL

All lanes:

Human kidney tissue lysate at 15 µg

Predicted band size: 142 kDa

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Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

Reacts with

Mouse, Human

Applications

ICC/IF, IHC-P, WB

applications

Immunogen

Synthetic Peptide within Human NPC intracellular cholesterol transporter 1. The exact immunogen used to generate this antibody is proprietary information.

O15118

Specificity

Will not cross-react with NPC2

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1-2 µg/mL", "WB-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" }, "Mouse": { "WB-species-checked": "guaranteed", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "guaranteed", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" } } }

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification Immunogen

Storage buffer

pH: 7.2 Preservative: 0.02% Sodium azide Constituents: PBS

Shipped at conditions

Blue Ice

Appropriate short-term storage duration

Up to 12 months

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

+4°C

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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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Product protocols

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

Visit the General protocols
Visit the Troubleshooting

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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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Publications (3)

Recent publications for all applications. Explore the full list and refine your search

Cells 12: PubMed36831222

2023

CXXC5 Mediates DHT-Induced Androgenetic Alopecia via PGD.

Applications

Unspecified application

Species

Unspecified reactive species

Yeong Chan Ryu,Jiyeon Park,You-Rin Kim,Sehee Choi,Geon-Uk Kim,Eunhwan Kim,Yumi Hwang,Heejene Kim,Gyoonhee Han,Soung-Hoon Lee,Kang-Yell Choi

Nature biotechnology 40:885-895 PubMed35190686

2022

Saturation variant interpretation using CRISPR prime editing.

Applications

Unspecified application

Species

Unspecified reactive species

Steven Erwood,Teija M I Bily,Jason Lequyer,Joyce Yan,Nitya Gulati,Reid A Brewer,Liangchi Zhou,Laurence Pelletier,Evgueni A Ivakine,Ronald D Cohn

Genome research 29:2010-2019 PubMed31754021

2019

Modeling Niemann-Pick disease type C in a human haploid cell line allows for patient variant characterization and clinical interpretation.

Applications

Unspecified application

Species

Unspecified reactive species

Steven Erwood,Reid A Brewer,Teija M I Bily,Eleonora Maino,Liangchi Zhou,Ronald D Cohn,Evgueni A Ivakine

View all publications

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Product promise

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For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

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Anti-Niemann Pick C1 antibody

Western blot - Anti-Niemann Pick C1 antibody (AB106534)

Western blot - Anti-Niemann Pick C1 antibody (AB106534)

Key facts

Host species

Clonality

Isotype

Carrier free

Reacts with

Applications

Immunogen

Specificity

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Reactivity data

Properties and storage information

Form

Purification technique

Storage buffer

Shipped at conditions

Appropriate short-term storage duration

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (3)

Product promise