Anti-Niemann Pick C2/NPC2 Antibody Picoband®

About this product

Boster Bio Anti-Niemann Pick C2/NPC2 Antibody Picoband® catalog # A01582-2. Tested in IHC, WB applications. This antibody reacts with Human. 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

A01582-2

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

NPC2 is a protein associated with Niemann-Pick disease, type C. This gene is mapped to chromosome 14q24.3. It 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

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

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence at the C-terminus of human Niemann Pick C2, which shares 79.4% and 76.5% amino acid (aa) sequence identity with mouse and rat Niemann Pick C2, respectively.

Clonality

Polyclonal

Tissue Specificity

Epididymis.

Applications

IHC, 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 endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. 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. Klunemann, H. H., Elleder, M., Kaminski, W. E., Snow, K., Peyser, J. M., O'Brien, J. F., Munoz, D., Schmitz, G., Klein, H. E., Pendlebury, W. W. Frontal lobe atrophy due to a mutation in the cholesterol binding protein HE1/NPC2. Ann. Neurol. 52: 743-749, 2002.

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

16 kDa

Specificity

No cross reactivity with other proteins.

Gene Name Synonym

NPC intracellular cholesterol transporter 2

Subcellular Location

Secreted.

Isotype

Rabbit IgG

Contents

Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.