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NPC1 Antibody

CAT: 0223-5185-01Size: 0.02 mgDry Ice: NoHazardous: No
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CAT#:0223-5185-01Size:0.02 mg
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Background
NPC1 Antibody: 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.
NCBI Gene ID
4864
Swiss Prot
O15118
Accession Number
NP_000262
Host
Rabbit
Reactivity
Human, Mouse
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Obesity
Purification
NPC1 Antibody is affinity chromatography purified via peptide column.
Positive Control
Cat. No. 1211 - HepG2 Cell Lysate
Concentration
1 mg/mL
Homology
Predicted species reactivity based on immunogen sequence: Pig: (87%)
Buffer
NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
NPC1 antibody can be stored at 4˚ C, stable for up to one year. Antibodies should not be exposed to prolonged high temperatures.
Fragment
IgG
Symbol
NPC1
NCBI Official Name
Niemann-Pick disease, type C1
NCBI Organism
Homo sapiens
Background Reference 01
Karten B, Peake KB, and Vance JE. Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells. Biochim. Biophys. Acta2009; 1791:656-70.
Background Reference 02
Carstea ED, Polymeropoulos MH, Parker CC, et al. Linkage of Niemann-Pick disease type C to human chromosome 18. Proc. Natl. Acad. Sci. USA1993; 90:2002-4.
Background Reference 03
Carstea ED, Morris JA, Coleman KG, et al. Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science1977; 277:228-31.
Background Reference 04
Garver WS and Heidenreich RA. The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system. Curr. Mol. Med.2002; 2:485-505.
Other Product Names
NPC1 Antibody: NPC, Niemann-Pick C1 protein
Tested Applications
ELISA, WB, IHC-P, IF
Protein ID
255652944
Physical Properties
Liquid

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