Antibody data
- Antibody Data
- Antigen structure
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- Product number
- abx015944 - Provider product page
- Provider
- Abbexa
- Product name
- NPC Intracellular Cholesterol Transporter 1 (NPC1) Antibody
- Antibody type
- Monoclonal
- Description
- 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.
- Reactivity
- Human
- Host
- Mouse
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 100 µl
- Concentration
- 1 mg/ml
- Storage
- Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
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