Anti-Niemann Pick C1/NPC1 Antibody Picoband®

About this product

Boster Bio Anti-Niemann Pick C1/NPC1 Antibody Picoband® catalog # A00428-2. Tested in ELISA, WB applications. This antibody reacts with Human, Mouse, Rat. 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

A00428-2

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11) . This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

Synonyms

Niemann-Pick C1 protein; NPC1

Gene Name

NPC1

Gene ID

4864

UniProt

O15118

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E. coli-derived human Niemann Pick C1 recombinant protein (Position: A1022-F1278) .

Clonality

Polyclonal

Applications

ELISA, WB

Field of Research

Apoptosis, Cancer, Cell Death, Cytokines, Growth Factors, Growth Factors/Hormones, Immunology, Innate Immunity, Invasion/Microenvironment, Receptors, Signal Transduction, TNF Superfamily

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 NPC2 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. 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.

References & Citations

1. Entrez Gene: NPC1 Niemann-Pick disease, type C1. 2. Carstea ED, Polymeropoulos MH, Parker CC, Detera-Wadleigh SD, O'Neill RR, Patterson MC, Goldin E, Xiao H, Straub RE, Vanier MT (March 1993) . Linkage of Niemann-Pick disease type C to human chromosome 18. Proc. Natl. Acad. Sci. U.S.A. 90 (5) : 2002–4.

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

170 kDa

Specificity

No cross reactivity with other proteins.

Gene Name Synonym

NPC intracellular cholesterol transporter 1

Subcellular Location

Late endosome membrane.

Isotype

Rabbit IgG

Contents

243