Recombinant
RabMAb

Recombinant Anti-Niemann Pick C1 antibody [EPR5209] (Allophycocyanin) (ab223985)

Overview

  • Product name

    Anti-Niemann Pick C1 antibody [EPR5209] (Allophycocyanin)
    See all Niemann Pick C1 primary antibodies
  • Description

    Rabbit monoclonal [EPR5209] to Niemann Pick C1 (Allophycocyanin)
  • Host species

    Rabbit
  • Conjugation

    Allophycocyanin. Ex: 645nm, Em: 660nm
  • Tested applications

    Suitable for: Flow Cytmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    corresponding to Human Niemann Pick C1 aa 1250-1350 (C terminal).
    Database link: O15118

  • Positive control

    • Flow Cyt: HepG2 cells
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

    This product is a recombinant rabbit monoclonal antibody.

Applications

Our Abpromise guarantee covers the use of ab223985 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
Flow Cyt 1/500.

The cellular localisation of this product has been verified in ICC/IF.

Target

  • Function

    Involved 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 disease

    Defects 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 similarities

    Belongs to the patched family.
    Contains 1 SSD (sterol-sensing) domain.
  • Domain

    A 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
    modifications

    Glycosylated.
  • Cellular localization

    Late endosome membrane. Lysosome membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • Niemann Pick C1 protein precursor antibody
    • Niemann Pick disease, type C1 antibody
    • Niemann-Pick C1 protein antibody
    • NPC antibody
    • NPC1 antibody
    • NPC1_HUMAN antibody
    see all

Images

  • Overlay histogram showing HepG2 cells stained with ab223985 (red line). The cells were fixed with 4% formaldehyde (10 min) and then permeabilized with 0.1% PBS-Triton X-100 for 15 min. The cells were then incubated in 1x PBS / 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (ab223985, 1/500 dilution) for 30 min at 22°C.

    Isotype control antibody (black line) was Rabbit IgG (monoclonal) Allophycocyanin (ab232814) used at the same concentration and conditions as the primary antibody. Unlabelled sample (blue line) was also used as a control.

    Acquisition of >5,000 events were collected using a 40 mW Red laser (640nm) and 670/14 bandpass filter.

    This antibody gave a positive signal in HepG2 cells fixed with 80% methanol (5 min)/permeabilized with 0.1% PBS-Triton X-100 for 15 min used under the same conditions.

References

ab223985 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab223985.
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