Niemann Pick C1 (NPC1) Rabbit Polyclonal Antibody
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Specifications
Product Data | |
Applications | ELISA, IF, IHC, WB |
Recommended Dilution | NPC1 antibody can be used for detection of NPC1 by Western blot at 1 µg/mL. Antibody can also be used for immunohistochemistry starting at 2.5 µg/mL. For immunofluorescence start at 20 µg/mL. Antibody validated: Western Blot in human samples; Immunohistochemistry in mouse samples and Immunofluorescence in mouse samples. All other applications and species not yet tested. |
Reactivities | Human, Mouse |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | NPC1 antibody was raised against a 16 amino acid peptide from near the carboxy terminus of human NPC1. |
Formulation | PBS containing 0.02% sodium azide. |
Concentration | 1ug/ul |
Purification | NPC1 Antibody is affinity chromatography purified via peptide column. |
Conjugation | Unconjugated |
Storage | Antibody can be stored at 4°C up to one year. Antibodies should not be exposed to prolonged high temperatures. |
Stability | Stable for 12 months from date of receipt. |
Gene Name | NPC intracellular cholesterol transporter 1 |
Database Link | |
Background | 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 hydrolized 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. |
Synonyms | NPC |
Reference Data | |
Protein Families | Druggable Genome, Transmembrane |
Protein Pathways | Lysosome |
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