Anti-Niemann Pick C2/NPC2 Picoband® Antibody

About this product

Boster Bio Anti-Niemann Pick C2/NPC2 Picoband® Antibody catalog # A01582-3. Tested in ELISA, Flow Cytometry, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Specifications

Catalog number

A01582-3

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

Epididymal secretory protein E1 (also called NPC intracellular cholesterol transporter 2, or NPC2) is a protein associated with Niemann-Pick disease, type C. It is mapped to 14q24.3. This gene encodes a protein containing a lipid recognition domain. The encoded protein may function in regulating the transport of cholesterol through the late endosomal/lysosomal system. Mutations in this gene have been associated with Niemann-Pick disease, type C2 and frontal lobe atrophy.

Synonyms

NPC intracellular cholesterol transporter 2; Epididymal secretory protein E1; Human epididymis-specific protein 1; He1; Niemann-Pick disease type C2 protein; NPC2; HE1

Gene Name

NPC2

Gene ID

10577

UniProt

P61916

Host

Rabbit

Reactivity

Human, Mouse

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E.coli-derived human Niemann Pick C2/NPC2 recombinant protein (Position: E20-L151) .

Clonality

Polyclonal

Tissue Specificity

Ubiquitously expressed.

Applications

ELISA, Flow Cytometry, IF, IHC, ICC, WB

Field of Research

Cardiovascular, Cholesterol Metabolism, Energy Metabolism, Energy Transfer Pathways, Lipid and Lipoprotein Metabolism, Lipid Metabolism, Lipids/Lipoproteins, Metabolic Signaling Pathways, Metabolism, Neurodegenerative Disease, Neurology Process, Neuroscience, Pathways and Processes, Signal Transduction

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the lysosomal compartment. 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. May bind and mobilize cholesterol that is associated with membranes. NPC2 binds cholesterol with a 1:1 stoichiometry. Can bind a variety of sterols, including lathosterol, desmosterol and the plant sterols stigmasterol and beta-sitosterol. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.

References & Citations

1. Blom, T. S., Linder, M. D., Snow, K., Pihko, H., Hess, M. W., Jokitalo, E., Veckman, V., Syvanen, A.-C., Ikonen, E. Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease. Hum. Molec. Genet. 12: 257-272, 2003. 2. Sleat, D. E., Wiseman, J. A., El-Banna, M., Price, S. M., Verot, L., Shen, M. M., Tint, G. S., Vanier, M. T., Walkley, S. U., Lobel, P. Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport. Proc. Nat. Acad. Sci. 101: 5886-5891, 2004. 3. Verot, L., Chikh, K., Freydiere, E., Honore, R., Vanier, M. T., Millat, G. Niemann-Pick C disease: functional characterization of three NPC2 mutations and clinical and molecular update on patients with NPC2. Clin. Genet. 71: 320-330, 2007.

Storage Conditions

Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Observed Molecular Weight

19 kDa

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

NPC intracellular cholesterol transporter 2

Subcellular Location

Secreted. Endoplasmic reticulum. Lysosome.

Isotype

Rabbit IgG

Contents

243