Anti-Niemann Pick C1 antibody (ab36983)

Overview

• Product nameAnti-Niemann Pick C1 antibodySee all Niemann Pick C1 primary antibodies ...
• Description
  Rabbit polyclonal to Niemann Pick C1
• Specificityab36983 recognises heterogeneously glycosylated Niemann Pick C1 protein.
• Tested applicationsElectron Microscopy, WB, ICC/IF, IP more details
• Species reactivity
  Reacts with: Mouse, Rat, Hamster, Human
  
• Immunogen

  Synthetic peptide made to the C terminal region of human Niemann Pick C1.

• Positive controlHuman fibroblast cell lysate

Properties

• FormLiquid
• Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
• Storage bufferPreservative: 0.025% Sodium Azide
  Constituents: PBS
• Concentration information loading...
• PurityProtein A purified
• Clonality Polyclonal
• IsotypeIgG
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

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

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

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

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

  • Metabolism
  • Types of disease
  • Heart disease

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

  • Cardiovascular
  • Lipids / Lipoproteins
  • Fatty Acids
  • Metabolism

  • Cardiovascular
  • Lipids / Lipoproteins
  • Lipid Metabolism
  • Cholesterol 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
  • Entrez Gene: 18145 Mouse
  • Entrez Gene: 266732 Rat
  • Omim: 607623 Human
  • SwissProt: O15118 Human
  • SwissProt: O35604 Mouse
  • Unigene: 464779 Human
  • Unigene: 715623 Human

  • Unigene: 3484 Mouse

  see all

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

  Niemann-Pick C1 protein antibodyNPC antibodyNPC1 antibodyNPC1_HUMAN antibody

  see all

Anti-Niemann Pick C1 antibody images

• 

  Immunocytochemistry/ Immunofluorescence - Niemann Pick C1 antibody (ab36983)This image was kindly supplied by Daniel Rodriguez by Abreview
  ab36983 at a 1/800 dilution staining Niemann Pick C1 in mouse 3T3-L1 cells by Immunocytochemistry/ Immunofluorescence, incubated for 12 hours at 4°C. Fixed in methanol. Blocked with 1% BSA for 1 hour 30 minutes at room temperature. Secondary used at 1/800 dilution monoclonal Goat anti-rabbit conjugated to Alexa Fluor 568 (red). Nucleus was stained with DAPI (blue).
  
  Note localization of Niemann Pick C1 around the nucleus and in cytosolic vesicles.

  See Abreview

• 

  Western blot - Niemann Pick C1 antibody (ab36983)
  
  
  Predicted band size : 142 kDa
  Observed band size : 180 kDa (why is the actual band size different from the predicted?)
  

  This image is courtesy of an anonymous Abreview

  See Abreview