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|Tested species reactivity||Human, Mouse|
|Host / Isotype||Rabbit / IgG|
|Immunogen||A 16 amino acid peptide from near the carboxy terminus of human NPC1.|
|Purification||Antigen affinity chromatography|
|Contains||0.02% sodium azide|
|Storage Conditions||Maintain refrigerated at 2-8°C for up to 3 months. For long term storage store at -20°C|
|Tested Applications||Dilution *|
|Immunocytochemistry (ICC)||20 ug/ml|
|Immunofluorescence (IF)||20 ug/ml|
|Immunohistochemistry (IHC)||2.5 µg/ml|
|Western Blot (WB)||1 µg/ml|
* 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.
A suggested positive control is human kidney tissue lysate.
PA5-20753 can be used with blocking peptide PEP-0867.
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.
Niemann Pick type C1; Niemann-Pick C1 protein; Niemann-Pick disease, type C1; NPC; sphingomyelinosis; truncated Niemann-Pick C1
A430089E03Rik; C85354; D18Ertd139e; D18Ertd723e; lcsd; nmf164; NPC; NPC1; spm