Product Specifications | |
---|---|
Species Reactivity |
Human |
Host/Isotype |
Rabbit / IgG |
Class |
Recombinant Monoclonal |
Type |
Antibody |
Clone |
JB87-33 |
Immunogen |
Recombinant protein within Human Niemann Pick C1 aa 1,160-1,278 |
Conjugate |
Unconjugated |
Form |
Liquid |
Concentration |
1 mg/mL |
Purification |
Protein A |
Storage buffer |
TBS, pH 7.4, with 40% glycerol, 0.05% BSA |
Contains |
0.05% sodium azide |
Storage conditions |
-20° C, Avoid Freeze/Thaw Cycles, store in dark |
RRID |
AB_2848602 |
Positive Control: SiHa, A549, HepG2, PC-3M, Rat kidney tissue, human kidney tissue, mouse testis tissue, SH-SY-5Y.
Mutations in the Niemann-Pick disease type C1 (NPC1) gene result in a fatal progressive neurodegenerative disorder characterized by an abnormal sequestration of lipids including cholesterol and glycosphingolipids. The NPC1 protein is 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. NPC1 transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolyzed and released as free cholesterol. NPC1, in addition to FTO, MC4R, and PTER has recently been shown to be a new risk loci for early-onset and morbid adult obesity in European populations. This anti-NPC1 antibody will not cross-react to NPC2, another gene whose defects also result in Niemann-Pick type C disease.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: FLJ98532; Niemann-Pick C1 protein; Niemann-Pick disease, type C1; NPC intracellular cholesterol transporter 1; truncated Niemann-Pick C1
Gene Aliases: NPC; NPC1
UniProt ID: (Human) O15118
Entrez Gene ID: (Human) 4864
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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