Niemann Pick C1 Antibody / NPC1

• Description:

  Niemann-Pick disease, type C1 (NPC1) is a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans it is encoded by the NPC1 gene (chromosome location 18q11). This gene encodes 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. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-absorbance lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

• UniProt:

  O15118

• Host:

  Rabbit

• Immunogen:

  Amino acids YHTVLQTSADFIDALKKARLI were used as the immunogen for the Niemann Pick C1 antibody.

• Clonality:

  Polyclonal

• Isotype:

  IgG

• Applications:

  WB, FACS

• Format:

  Antigen affinity purified

• Buffer:

  0.5mg/ml if reconstituted with 0.2ml sterile DI water

• Reconstitution:

  After reconstitution, the Niemann Pick C1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

• Limitations:

  This Niemann Pick C1 antibody is available for research use only.

• CAS Number:

  9007-83-4