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039083-APC

NPC1, ID (NPC1, Niemann-Pick C1 protein) (APC)

Size

£889.00

SKU:
039083-APC
Purity:
Purified by Protein A and peptide affinity chromatography
Storage Conditions:
4°C Do Not Freeze
Supplier:
United States Biological
Host:
Rabbit
Immunogen:
KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 591-620 in the central region of human NPC1
Format:
Supplied as a liquid in PBS, pH 7.2. Labeled with Allophycocyanin (APC).
Extra Details:
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. Applications: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence and FLISA. Other applications have not been tested. Recommended Dilutions: Immunohistochemistry: Formalin-fixed, paraffin-embedded sections Optimal dilutions to be determined by the researcher. Storage and Stability: Store product at 4°C in the dark. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Note: Applications are based on unconjugated antibody.
Shipping Conditions:
Blue Ice