NPC1 polyclonal antibody
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Specification
Product Description
Rabbit polyclonal antibody raised against synthetic peptide of NPC1.
Immunogen
A synthetic peptide corresponding to amino acids 1230-1278 at C-terminus of human NPC1.
Host
Rabbit
Reactivity
Hamster, Human, Mouse
Specificity
This antibody is specific to NPC1 protein.
Form
Liquid
Recommend Usage
Immunofluorescence (1:250)
Western Blot (1:1000-1:3000)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.09% sodium azide)
Storage Instruction
Store at 4°C for short term. 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 (Cell lysate)
Western blot analysis of NPC1 in 20 ug of human fibroblast cell lysate with NPC1 polyclonal antibody (Cat # PAB12093).Immunohistochemistry
Immunofluorescence
Immunoprecipitation
Immunoelectron Microscopy
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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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Interactome
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Pathway
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Disease
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Publication Reference
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Primary Cilium Alterations and Expression Changes of Patched1 Proteins in Niemann-pick Type C Disease.
Formichi P, Battisti C, De Santi MM, Guazzo R, Tripodi SA, Radi E, Rossi B, Tarquini E, Federico A.
Amino Acids 2018 Jan; 233(1):663.
Application:WB-Ce, Human, Human skin fibroblasts.
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Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the golden Syrian hamster.
Burke KT, Colvin PL, Myatt L, Graf GA, Schroeder F, Woollett LA.
Journal of Lipid Research 2009 Jan; 50(6):1146.
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Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function.
Frolov A, Srivastava K, Daphna-Iken D, Traub LM, Schaffer JE, Ory DS.
The Journal of Biological Chemistry 2001 Dec; 276(49):46414.
Application:ICC, IEM, IF, Mouse, CHO, M87 cells.
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Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis.
Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS.
The Journal of Biological Chemistry 2000 Dec; 275(49):38445.
Application:IF, WB-Tr, Mouse, CHO cells.
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Primary Cilium Alterations and Expression Changes of Patched1 Proteins in Niemann-pick Type C Disease.
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