NPC1 monoclonal antibody, clone 8D10B6
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Specifications
Product Description
Mouse monoclonal antibody raised against recombinant human NPC1.
Immunogen
Recombinant protein corresponding to amino acids 34-174 of human NPC1 from E. coli.
Host
Mouse
Theoretical MW (kDa)
142.2
Reactivity
Human
Form
Liquid
Isotype
IgG1
Recommend Usage
ELISA (1:10000)
Immunohistochemistry (1:200-1:1000)
Western Blot (1:500-1:2000)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.05% sodium azide).
Storage Instruction
Store at 4°C. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Transfected lysate)
Western blot analysis of (1) HEK293 cells, (2) NPC1-hIgGFc transfected HEK293 cell lysate with NPC1 monoclonal antibody.Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining of paraffin-embedded esophageal cancer tissue with NPC1 monoclonal antibody.Enzyme-linked Immunoabsorbent Assay
ELISA analysis of NPC1 monoclonal antibody, clone 8D10B6. -
Gene Info — NPC1
Entrez GeneID
4864Gene Name
NPC1
Gene Alias
NPC
Gene Description
Niemann-Pick disease, type C1
Gene Ontology
HyperlinkGene Summary
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
Other Designations
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