Anti-Niemann Pick C1/NPC1 Antibody Picoband™ | Boster A00428-2 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

Boster

product type :

antibody

product name :

Anti-Niemann Pick C1/NPC1 Antibody Picoband™

catalog :

A00428-2

quantity :

100µg/vial

clonality :

polyclonal

host :

domestic rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot, ELISA

product information

SKU :

A00428-2

Product Name :

Anti-Niemann Pick C1/NPC1 Antibody Picoband™

Size :

100µg/vial

Clonality :

Polyclonal

Host :

Rabbit

Reactivity :

Human, Mouse, Rat

Application(s) :

ELISA, WB

Application Details :

Western blot,0.1-0.5µg/ml. Direct ELISA,0.1-0.5µg/ml.

Description :

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

Concentration :

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

Gene Name :

NPC1

Uniprot ID :

O15118

Immunogen :

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

Form :

Lyophilized

Contents :

Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na 2 HPO 4 , 0.05mg NaN 3 .

Cross-reactivity :

No cross reactivity with other proteins.

Storage :

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.

Reconstitution :

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

Gene Full Name :

NPC intracellular cholesterol transporter 1

Synonyms :

Niemann-Pick C1 protein; NPC1

Protein 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.

Subcellular Localization :

Late endosome membrane.

Recommended Detection Systems :

Boster recommends Enhanced Chemiluminescent Kit with anti-Rabbit IgG (EK1002) for Western blot.

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.