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|Tested species reactivity||Human|
|Published species reactivity||Human|
|Host / Isotype||Mouse / IgG1|
|Immunogen||Fragments of Human NPC1 conjugated to a carrier protein.|
|Storage buffer||PBS, pH 7.4|
|Contains||0.1% sodium azide|
|Tested Applications||Dilution *|
|ELISA (ELISA)||Assay Dependent|
|Immunocytochemistry (ICC)||Assay Dependent|
|Immunofluorescence (IF)||Assay Dependent|
|Western Blot (WB)||Assay Dependent|
* Suggested working dilutions are given as a guide only. It is recommended that the user titrate the product for use in their own experiment using appropriate negative and positive controls.
|Western Blot (WB)||See 2 publications below|
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
|Human||Not Cited||Cholesterol as a factor regulating intracellular localization of annexin A6 in Niemann-Pick type C human skin fibroblasts.||Sztolsztener ME,Strzelecka-Kiliszek A,Pikula S,Tylki-Szymanska A,Bandorowicz-Pikula J||Archives of biochemistry and biophysics (493:221)||2010|
|Human||Not Cited||NO-1886 up-regulates Niemann-Pick C1 protein (NPC1) expression through liver X receptor alpha signaling pathway in THP-1 macrophage-derived foam cells.||Ma X,Hu YW,Mo ZC,Li XX,Liu XH,Xiao J,Yin WD,Liao DF,Tang CK||Cardiovascular drugs and therapy / sponsored by the International Society of Cardiovascular Pharmacotherapy (23:199)||2009|
Niemann-Pick disease, type C1; NPC; truncated Niemann-Pick C1