NPC1 Antibody / Niemann Pick C1
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-density 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.
Product Specifications
CAS Number
9007-83-4
Specifications
Western blot: 1:1000, Immunofluorescence: 1:10-1:50, Immunohistochemistry (Paraffin) : 1:10-1:50
UniProt
O15118
Host
Rabbit
Reactivity
Human
Immunogen
A portion of amino acids 591-620 from the human protein was used as the immunogen for this NPC1 antibody.
Clonality
Polyclonal
Isotype
Ig
Applications
IF, IHC, WB, ELISA
Purity
Antigen affinity
Format
Antigen affinity purified
Buffer
In 1X PBS, pH 7.4, with 0.09% sodium azide
Reconstitution
Aliquot the NPC1 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Limitations
This NPC1 antibody is available for research use only.
Storage Conditions
Aliquot the NPC1 antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
Formulation
In 1X PBS, pH 7.4, with 0.09% sodium azide
Applications Notes
Titration of the NPC1 antibody may be required due to differences in protocols and secondary/substrate sensitivity.
Image Legend
NPC1 antibody western blot analysis in NCI-H460 lysate. Predicted molecular weight ~142/170~190 kDa (unmodified/glycosylated) .
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