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

Scan QR to view Datasheet Catalog #    MBS150200
Unit / Price
0.1 mg  /  $365 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

NPC1, Polyclonal Antibody

 Also Known As   

NPC1 Antibody

 Product Synonym Names    NPC1; NPC; Niemann-Pick C1 protein; Niemann-Pick disease, type C1
 Product Gene Name   

anti-NPC1 antibody

[Similar Products]
 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.
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 OMIM    phenotype 607623
 3D Structure    ModBase 3D Structure for O15118
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
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 Purity/Purification    NPC1 Antibody is affinity chromatography purified via peptide column.
 Form/Format    Liquid
 Concentration    1 mg/mL (lot specific)
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 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.
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 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.
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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.
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 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
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 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
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 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
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NCBI/Uniprot data below describe general gene information for NPC1. It may not necessarily be applicable to this product.
 NCBI GI #    255652944
 NCBI GeneID    4864
 NCBI Accession #    NP_000262 [Other Products]
 NCBI GenBank Nucleotide #    NM_000271.4 [Other Products]
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 UniProt Primary Accession #    O15118 [Other Products]
 UniProt Secondary Accession #    Q9P130; B4DET3 [Other Products]
 UniProt Related Accession #    O15118 [Other Products]
 Molecular Weight    107,025 Da
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 NCBI Official Full Name    Niemann-Pick C1 protein
 NCBI Official Synonym Full Names    Niemann-Pick disease, type C1
 NCBI Official Symbol    NPC1 [Similar Products]
 NCBI Official Synonym Symbols   
NPC
[Similar Products]
 NCBI Protein Information    Niemann-Pick C1 protein
 UniProt Protein Name    Niemann-Pick C1 protein
 Protein Family    Niemann-Pick C1 protein
 UniProt Gene Name    NPC1 [Similar Products]
 UniProt Entry Name    NPC1_HUMAN
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 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]
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 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
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 Research Articles on NPC1    1. Data suggest that in order for the ligand cholesterol to slide from one binding pocket to the other (from NPC2 to NPC1), cholesterol undergoes conformational change/isomerization to accommodate the bent transfer pathway between the 2 binding pockets.
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 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.
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Products associated with anti-NPC1 antibodyPathways associated with anti-NPC1 antibody
 Reference Product  PubMed Publications
 NPC2 antibody  >228 publications with NPC1 and NPC2
 LRP6 antibody  >2 publications with NPC1 and LRP6
 ABCG4 antibody  >2 publications with NPC1 and ABCG4
 UBC antibody  >2 publications with NPC1 and UBC
 TMEM97 antibody  >1 publications with NPC1 and TMEM97
 Products by Pathway  Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
Diseases associated with anti-NPC1 antibodyOrgans/Tissues associated with anti-NPC1 antibody
 Disease Name  Pubmed Publications
 Niemann-Pick Disease, Type C Antibodies  >204 publications with NPC1 and Niemann-Pick Disease, Type C
 Disease Models, Animal Antibodies  >113 publications with NPC1 and Disease Models, Animal
 Liver Diseases Antibodies  >58 publications with NPC1 and Liver Diseases
 Ataxia Antibodies  >40 publications with NPC1 and Ataxia
 Nerve Degeneration Antibodies  >37 publications with NPC1 and Nerve Degeneration
 Obesity Antibodies  >28 publications with NPC1 and Obesity
 Atherosclerosis Antibodies  >27 publications with NPC1 and Atherosclerosis
 Inflammation Antibodies  >24 publications with NPC1 and Inflammation
 Dysarthria Antibodies  >16 publications with NPC1 and Dysarthria
 Fatty Liver Antibodies  >15 publications with NPC1 and Fatty Liver
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >202 publications with NPC1 and Brain
 Liver Antibodies  >124 publications with NPC1 and Liver
 Blood Antibodies  >83 publications with NPC1 and Blood
 Ovary Antibodies  >40 publications with NPC1 and Ovary
 Skin Antibodies  >29 publications with NPC1 and Skin
 Lung Antibodies  >23 publications with NPC1 and Lung
 Spleen Antibodies  >23 publications with NPC1 and Spleen
 Bone Antibodies  >16 publications with NPC1 and Bone
 Vascular Antibodies  >16 publications with NPC1 and Vascular
 Bone Marrow Antibodies  >15 publications with NPC1 and Bone Marrow
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