NPC1 Antibody

• Background :

  NPC1 Antibody: 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.

• NCBI Gene ID :

  4864

• Swiss Prot :

  O15118

• Accession Number :

  NP_000262

• Host :

  Rabbit

• Reactivity :

  Human, Mouse

• Clonality :

  Polyclonal

• Conjugation :

  Unconjugated

• Type :

  Primary Antibodies

• Field of Research :

  Obesity

• Purification :

  NPC1 Antibody is affinity chromatography purified via peptide column.

• Positive Control :

  Cat. No. 1211 - HepG2 Cell Lysate

• Concentration :

  1 mg/mL

• Homology :

  Predicted species reactivity based on immunogen sequence: Pig: (87%)

• Buffer :

  NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.

• Modification :

  None

• Shipping Conditions :

  Blue Ice

• Storage Conditions :

  NPC1 antibody can be stored at 4˚ C, stable for up to one year. Antibodies should not be exposed to prolonged high temperatures.

• Fragment :

  IgG

• Symbol :

  NPC1

• NCBI Official Name :

  Niemann-Pick disease, type C1

• NCBI Organism :

  Homo sapiens

• Background Reference 01 :

  Karten B, Peake KB, and Vance JE. Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells. Biochim. Biophys. Acta2009; 1791:656-70.

• Background Reference 02 :

  Carstea ED, Polymeropoulos MH, Parker CC, et al. Linkage of Niemann-Pick disease type C to human chromosome 18. Proc. Natl. Acad. Sci. USA1993; 90:2002-4.

• Background Reference 03 :

  Carstea ED, Morris JA, Coleman KG, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science1977; 277:228-31.

• Background Reference 04 :

  Garver WS and Heidenreich RA. The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system. Curr. Mol. Med.2002; 2:485-505.

• Other Product Names :

  NPC1 Antibody: NPC, Niemann-Pick C1 protein

• Tested Applications :

  ELISA, WB, IHC-P, IF

• Protein ID :

  255652944

• Physical Properties :

  Liquid