Niemann Pick C1 (NPC1) Rabbit Polyclonal Antibody

CAT#: TA306785

Rabbit Polyclonal NPC1 Antibody

Product Images

Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 antibody at 1 ug/ml.

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  Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 antibody at 2.5 ug/ml.

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  Immunofluorescence of NPC1 in Mouse Kidney cells with NPC1 antibody at 20 ug/mL.

Specifications

Product Data

• Applications: IF, IHC, WB
• Recommended Dilution: WB: 1 ug/mL, ICC: 2.5 ug/mL, IF: 20 ug/mL
• Reactivities: Human
• 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: Affinity chromatography purified via peptide column
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Gene Name: NPC intracellular cholesterol transporter 1
• Database Link:
  NP_000262
  Entrez Gene 4864 Human
  UniProt ID O15118
• 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