Item no. |
BOS-A00428-2 |
Manufacturer |
Boster
|
Amount |
100 ug/vial |
Category |
|
Type |
Antibody Polyclonal |
Format |
Lyophilized |
Applications |
WB, ELISA |
Specific against |
Human, Mouse, Rat |
Host |
Rabbit |
Isotype |
IgG |
ECLASS 10.1 |
32160702 |
ECLASS 11.0 |
32160702 |
UNSPSC |
12352203 |
Alias |
Niemann-Pick C1 protein; NPC1 |
Available |
|
Manufacturers Product Category |
Primary Antibodies, Rabbit Polyclonal Antibodies |
Short 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. |
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. |
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. |
Gene Name |
NPC1 |
Application Details |
Western blot, 0.1-0.5μg/ml Direct ELISA, 0.1-0.5μg/ml |
Clonality |
Polyclonal |
Concentration |
Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml. |
Contents |
Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3. |
Immunogen |
E. coli-derived human Niemann Pick C1 recombinant protein (Position: A1022-F1278). |
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 |
Molecular Weight |
170 kDa |
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. |
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