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Applications | Tested Dilution | Publications |
---|---|---|
Western Blot (WB) |
0.5 µg/mL | - |
Product Specifications | |
---|---|
Species Reactivity |
Human |
Host/Isotype |
Goat / IgG |
Class |
Polyclonal |
Type |
Antibody |
Immunogen |
Peptide with sequence C-TYHTVLQTSADFID. |
Conjugate |
Unconjugated |
Form |
Liquid |
Concentration |
0.5 mg/mL |
Purification |
Antigen affinity chromatography |
Storage buffer |
TBS, pH 7.3, with 0.5% BSA |
Contains |
0.02% sodium azide |
Storage conditions |
-20° C, Avoid Freeze/Thaw Cycles |
This antibody is tested in Peptide ELISA: antibody detection limit dilution 1:128,000.
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) Q9P130
Entrez Gene ID: (Human) 4864
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