Niemann Pick C1 (NPC1) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, IF, IHC, WB
Recommend Dilution	NPC1 antibody can be used for detection of NPC1 by Western blot at 1 µg/mL. Antibody can also be used for immunohistochemistry starting at 2.5 µg/mL. For immunofluorescence start at 20 µg/mL. Antibody validated: Western Blot in human samples; Immunohistochemistry in mouse samples and Immunofluorescence in mouse samples. All other applications and species not yet tested.
Reactivity	Human, Mouse
Host	Rabbit
Clonality	Polyclonal
Immunogen	NPC1 antibody was raised against a 16 amino acid peptide from near the carboxy terminus of human NPC1.
Formulation	PBS containing 0.02% sodium azide.
Concentration	1ug/ul
Purification	NPC1 Antibody is affinity chromatography purified via peptide column.
Conjugation	Unconjugated
Storage Condition	Antibody can be stored at 4°C up to one year. Antibodies should not be exposed to prolonged high temperatures.
Gene Name	NPC intracellular cholesterol transporter 1
Database Link	NP_000262 Entrez Gene 18145 MouseEntrez Gene 4864 Human UniProt ID O15118
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.
Synonyms	NPC
Reference Data
Protein Families	Druggable Genome, Transmembrane
Protein Pathways	Lysosome

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