Anti-NPC1 Antibody

• 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.

• Description:

  Boster Bio Anti-NPC1 Antibody (Catalog # A00428) . Tested in ELISA, WB, IHC-P, IF applications. This antibody reacts with Human, Mouse.

• Synonyms:

  NPC, Niemann-Pick C1 protein

• Gene Name:

  NPC1

• UniProt:

  O15118

• Host:

  Rabbit

• Reactivity:

  Human, Mouse

• Immunogen:

  NPC1 antibody was raised against a 16 amino acid synthetic peptide from near the carboxy terminus of human NPC1. The immunogen is located within the last 50 amino acids of NPC1.

• Clonality:

  Polyclonal

• Applications:

  ELISA, WB, IHC-P, IF

• Field of Research:

  Obesity

• Purification:

  NPC1 Antibody is affinity chromatography purified via peptide column.

• Concentration:

  1 mg/mL

• Form:

  Liquid

• Function:

  Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment (PubMed:9211849, PubMed:9927649, PubMed:10821832, PubMed:18772377, PubMed:27238017, PubMed:12554680) . Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol- binding pocket in the N-terminal domain of NPC1 (PubMed:9211849, PubMed:9927649, PubMed:18772377, PubMed:19563754, PubMed:27238017, PubMed:28784760) . Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket (PubMed:19563754) . Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals (Probable) .

• Molecular Weight:

  142167 MW

• Shipping Conditions:

  Available

• Storage Conditions:

  NPC1 antibody can be stored at 4°C for three months and -20°C, stable for up to one year. Avoid repeated freeze-thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

• Fragment:

  IgG

• Other Gene Names:

  Niemann-Pick disease, type C1

• Subcellular Location:

  Late endosome membrane

• Prediction Reactivity:

  Pig

• Protein Name:

  Niemann-Pick disease, type C1