NPC1 Antibody
Product Details
- Cat. No.
- 254076
- Type
- Primary Antibody
- Clonality
- Polyclonal
- Host
- Rabbit
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Description
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.
Biological Information
- Clonality: Polyclonal
- Host: Rabbit
- Reactivity: Human
- Antigen: KLH-conjugated synthetic peptide encompassing a sequence within human NPC1.
- Isotype: Rabbit Ig
- Gene: NP_000262
Handling
- Quantity: 0.1 mg
- Storage: Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Applications
- ELISA (ELISA)
- Immunohistochemistry (IHC)
- Western Blotting (WB)