NPC1 polyclonal antibody
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More Files
- More Functions
Western Blot (Tissue lysate)
Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 polyclonal antibody (Cat # PAB16750) at 1 ug/mL .
Immunohistochemistry
Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 polyclonal antibody (Cat # PAB16750) at 2.5 ug/mL .
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Specification
Product Description
Rabbit polyclonal antibody raised against synthetic peptide of NPC1.
Immunogen
A synthetic peptide corresponding to C-terminus 16 amino acids of human NPC1.
Host
Rabbit
Reactivity
Human
Form
Liquid
Recommend Usage
Western Blot (1-2 ug/mL)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.02% sodium azide)
Storage Instruction
Store at 4°C for three months. 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 (Tissue lysate)
Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 polyclonal antibody (Cat # PAB16750) at 1 ug/mL .Immunohistochemistry
Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 polyclonal antibody (Cat # PAB16750) at 2.5 ug/mL .Enzyme-linked Immunoabsorbent Assay
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Gene Info — NPC1
Entrez GeneID
4864Protein Accession#
NP_000262Gene 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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Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells.
Karten B, Peake KB, Vance JE.
Biochimica et Biophysica Acta 2009 Feb; 1791(7):659.
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The Niemann-Pick C proteins and trafficking of cholesterol through the late endosomal/lysosomal system.
Garver WS, Heidenreich RA.
Current Molecular Medicine 2002 Aug; 2(5):485.
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Linkage of Niemann-Pick disease type C to human chromosome 18.
Carstea ED, Polymeropoulos MH, Parker CC, Detera-Wadleigh SD, O'Neill RR, Patterson MC, Goldin E, Xiao H, Straub RE, Vanier MT, et al..
PNAS 1993 Mar; 90(5):2002.
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Mechanisms and consequences of impaired lipid trafficking in Niemann-Pick type C1-deficient mammalian cells.
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