NPC1 polyclonal antibody
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More Files
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining (Formalin-fixed paraffin-embedded sections) of human gall bladder with NPC1 polyclonal antibody (Cat # PAB30241) shows strong cytoplasmic positivity in glandular cells at 1:50 - 1:200 dilution.
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Specification
Product Description
Rabbit polyclonal antibody raised against recombinant human NPC1.
Immunogen
Recombinant protein corresponding to amino acids of human NPC1.
Sequence
VLNKVDIGLDQSLSMPDDSYMVDYFKSISQYLHAGPPVYFVLEEGHDYTSSKGQNMVCGGMGCNNDSLVQQIFNAAQLDNYTRIGFAPSSWIDDYFDWVKPQSSCCRVDNITDQFCNASVVD
Host
Rabbit
Reactivity
Human
Form
Liquid
Purification
Antigen affinity purification
Isotype
IgG
Recommend Usage
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (1:50-1:200)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS, pH 7.2 (40% glycerol, 0.02% 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
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunohistochemical staining (Formalin-fixed paraffin-embedded sections) of human gall bladder with NPC1 polyclonal antibody (Cat # PAB30241) shows strong cytoplasmic positivity in glandular cells at 1:50 - 1:200 dilution. -
Gene Info — NPC1
Entrez GeneID
4864Protein Accession#
O15118Gene 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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Interactome
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Pathway
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Disease
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