Anti-Niemann Pick C1 antibody (ab55706)

Overview

• Product nameAnti-Niemann Pick C1 antibodySee all Niemann Pick C1 primary antibodies ...
• Description
  Mouse monoclonal to Niemann Pick C1
• Tested applicationsWB, ICC, IHC-P, ICC/IF, Flow Cyt more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Recombinant fragment: GFANAMYNAC RDVEAPSSND KALGLLCGKD ADACNATNWI EYMFNKDNGQ APFTITPVFS DFPVHGMEPM NNATKGCDES VDEVTAPCSC QDCSIVCGPK , corresponding to amino acids 151-250 of Human Niemann Pick C1

  Run BLAST with Run BLAST with

Properties

• FormLiquid
• Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: None
  PBS, pH 7.2
• Concentration information loading...
• PurityProtein G purified
• Clonality Monoclonal
• IsotypeIgG2a
• Light chain typekappa
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Lipid and lipoprotein metabolism
  • Fatty acids

  • Metabolism
  • Pathways and Processes
  • Redox metabolism
  • Fatty acid oxidation

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Lipid and lipoprotein metabolism
  • Cholesterol Metabolism

  • Metabolism
  • Types of disease
  • Heart disease

  • Metabolism
  • Pathways and Processes
  • Metabolic signaling pathways
  • Lipid and lipoprotein metabolism
  • Lipoprotein metabolism

  • Signal Transduction
  • Protein Trafficking
  • Vesicle Transport
  • Coat Proteins

  • Cardiovascular
  • Lipids / Lipoproteins
  • Lipid Metabolism
  • Cholesterol Metabolism

  • Cardiovascular
  • Lipids / Lipoproteins
  • Fatty Acids
  • Metabolism

  • Cardiovascular
  • Atherosclerosis
  • Lipoprotein metabolism

  • Neuroscience
  • Neurotransmitter
  • Transporters
  • Other

  • Neuroscience
  • Neurology process
  • Neurodegenerative disease
  • Other

Applications

Target

• FunctionInvolved in the intracellular trafficking of 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.
• Involvement in diseaseDefects in NPC1 are the cause of Niemann-Pick disease type C1 (NPDC1) [MIM:257220]. 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.
• Sequence similaritiesBelongs to the patched family.
  Contains 1 SSD (sterol-sensing) domain.
• DomainA cysteine-rich N-terminal domain and a C-terminal domain containing a di-leucine motif necessary for lysosomal targeting are critical for mobilization of cholesterol from lysosomes.
• Post-translational
  modificationsGlycosylated.
• Cellular localizationLate endosome membrane. Lysosome membrane.

Target information above from: UniProt accession O15118 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 4864 Human
  • Omim: 607623 Human
  • SwissProt: O15118 Human
  • Unigene: 464779 Human
  • Unigene: 715623 Human

• Alternative names
  Niemann Pick C1 protein precursor antibodyNiemann Pick disease, type C1 antibodyNiemann-Pick C1 protein antibody

  NPC antibodyNPC1 antibodyNPC1_HUMAN antibody

  see all

Anti-Niemann Pick C1 antibody images

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  Western blot - Niemann Pick C1 antibody (ab55706)
  Western blot against tagged recombinant protein immunogen using ab55706 Niemann Pick C1 antibody at 1ug/ml. Predicted band size of immunogen is 34 kDa
• 

  Immunocytochemistry/ Immunofluorescence-Niemann Pick C1 antibody(ab55706)
  ab55706 at 10 ug/ml staining Niemann Pick C1 in human A-431 cells by Immunocytochemistry/ Immunofluorescence.
  
  
• 

  Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Niemann Pick C1 antibody (ab55706)
  IHC image of ab55706 staining in human normal liver formalin fixed paraffin embedded tissue section, performed on a Leica Bond^TM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab55706, 1µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
  
  For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
  
  
• 

  Flow Cytometry-Anti-Niemann Pick C1 antibody(ab55706)
  Overlay histogram showing A431 cells stained with ab55706 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab55706, 1µg/1x10⁶ cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 1µg/1x10⁶ cells) used under the same conditions. Acquisition of >5,000 events was performed.