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anti-NPC1 antibody :: Rabbit anti-Human NPC1 Polyclonal Antibody

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Catalog # MBS150200
Unit / Price
  0.1 mg  /  $375 +1 FREE 8GB USB
Product Name

NPC1, Polyclonal Antibody

Also Known As

NPC1 Antibody

Product Synonym Names
NPC1; NPC; Niemann-Pick C1 protein; Niemann-Pick disease, type C1
Antibody/Peptide Pairs
NPC1 peptide (MBS152485) is used for blocking the activity of NPC1 antibody (MBS150200)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
1278
OMIM
phenotype 607623
3D Structure
ModBase 3D Structure for O15118
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
NPC1 Antibody is affinity chromatography purified via peptide column.
Form/Format
Liquid
Concentration
1 mg/mL (lot specific)
Conjugate
Unconjugated
Immunogen
NPC1 antibody was raised against a 16 amino acid synthetic peptide from near the carboxy terminus of human NPC1.
Buffer
NPC1 Antibody is supplied in PBS containing 0.02% sodium azide.
Preparation and Storage
NPC1 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.
Other Notes
Small volumes of anti-NPC1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-NPC1 antibody
NPC1 Antibody: 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.
Applications Tested/Suitable for anti-NPC1 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
Application Notes for anti-NPC1 antibody
NPC1 antibody can be used for detection of NPC1 by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Western Blot (WB) of anti-NPC1 antibody
Western blot analysis of NPC1 in human kidney tissue lysate with NPC1 antibody at 1 μg/mL.
anti-NPC1 antibody Western Blot (WB) (WB) image
Immunohistochemistry (IHC) of anti-NPC1 antibody
Immunohistochemistry of NPC1 in mouse kidney tissue with NPC1 antibody at 2.5 μg/mL.
anti-NPC1 antibody Immunohistochemistry (IHC) (IHC) image
Immunofluorescence (IF) of anti-NPC1 antibody
Immunofluorescence of NPC1 in Mouse Kidney cells with NPC1 antibody at 20 μg/mL.
anti-NPC1 antibody Immunofluorescence (IF) image
NCBI/Uniprot data below describe general gene information for NPC1. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
107,025 Da
NCBI Official Full Name
Niemann-Pick C1 protein
NCBI Official Synonym Full Names
Niemann-Pick disease, type C1
NCBI Official Symbol
NCBI Official Synonym Symbols
NCBI Protein Information
Niemann-Pick C1 protein
UniProt Protein Name
Niemann-Pick C1 protein
Protein Family
UniProt Gene Name
UniProt Entry Name
NPC1_HUMAN
NCBI Summary for NPC1
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.[provided by RefSeq, Aug 2009]
UniProt Comments for NPC1
NPC1: Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals. Defects in NPC1 are the cause of Niemann-Pick disease type C1 (NPC1). A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C1 has a highly variable clinical phenotype. Clinical features include variable hepatosplenomegaly and severe progressive neurological dysfunction such as ataxia, dystonia and dementia. The age of onset can vary from infancy to late adulthood. An allelic variant of Niemann-Pick disease type C1 is found in people with Nova Scotia ancestry. Patients with the Nova Scotian clinical variant are less severely affected. Belongs to the patched family.

Protein type: Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 18q11.2

Cellular Component: Golgi apparatus; membrane; endoplasmic reticulum; late endosome membrane; perinuclear region of cytoplasm; lysosome; integral to plasma membrane; lysosomal membrane; integral to membrane; extracellular region; nuclear envelope; lipid raft

Molecular Function: protein binding; transmembrane receptor activity; sterol transporter activity; hedgehog receptor activity; cholesterol binding; receptor activity

Biological Process: response to drug; cholesterol metabolic process; lysosomal transport; cholesterol transport; bile acid metabolic process; protein amino acid glycosylation; cholesterol efflux; endocytosis; signal transduction; adult walking behavior; negative regulation of macroautophagy; cholesterol homeostasis; response to cadmium ion; autophagy; lipid raft organization and biogenesis

Disease: Niemann-pick Disease, Type C1
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Pathways associated with anti-NPC1 antibody
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