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, IPmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Hamster, Human
  • Immunogen

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

  • Positive control
    • Human fibroblast cell lysate

Applications

Our Abpromise guarantee covers the use of ab36983 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
Electron Microscopy
WB
ICC/IF
IP
  • Application notesElectron Microscopy: Use at an assay dependent dilution.
    ICC/IF: 1/250.
    IP: Use at an assay dependent dilution.
    WB: 1/1000 - 1/3000 using ECL technique. Predicted molecular weight: 142 kDa.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • 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
      modifications
      Glycosylated.
    • Cellular localizationLate endosome membrane. Lysosome membrane.
    • Information by UniProt
    • Database links
    • Alternative names
      • Niemann Pick C1 antibody
      • 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

    Anti-Niemann Pick C1 antibody images

    • 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



    • 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

    References for Anti-Niemann Pick C1 antibody (ab36983)

    This product has been referenced in:
    • Yu T  et al. Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts. Hum Mol Genet 21:3205-14 (2012). Read more (PubMed: 22505584) »
    • Ordonez MP  et al. Disruption and therapeutic rescue of autophagy in a human neuronal model of Niemann Pick type C1. Hum Mol Genet 21:2651-62 (2012). Read more (PubMed: 22437840) »

    See all 9 Publications for this product

    Product Wall

    Thank you for kindly confirming these details. I am sorry this vial of antibody has not worked for this customer.

    As requested, we are pleased to arrange a free of charge replacement. I can confirm that 1 vial of ab36983 has been added to yo...

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    Thank you for taking the time to complete our questionnaire and contact us. I am sorry to hear you have had difficulty obtaining satisfactory results from this antibody.

    The details you have kindly provided will provide us with vital informa...

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    Thank you for your inquiry.

    Unfortunately, I don't think that any of the http://www.abcam.com/index.html?t=158346&pt=1 we have in our catalog is suitable for the detection of the yeast Niemann-Pick type C-related protein 1 (http://www.unip...

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    I spoke with regarding Lisa regarding ab3698863.

    I don't believe I spoke with her about ab55706, however. What sort of trouble are you having with ab36983?

    Thanks for the additional information!

    Thanks for your reply.

    I agree there does seem to be an issue with this antibody. If you have purchased this vial of ab36983 within the past 6 months I am happy to offer a replacement, credit or refund. In your reply, please include your PO n...

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    Thanks for your reply.

    I'm sorry my initial questions were unclear. In question 1, I was wondering if any other samples, such as a positive control sample, had been blotted with ab55706 and ab36983.

    In question2 I was wondering if a...

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    Thank you for your enquiry and for providing all the protocoldetails. I am sorry to hear the vials of ab55706 and ab36983 you received are not working as expected. I just have a few additional questions:

    1. Have you used these antibodies to b...

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    Thank you for contacting us.

    I have been in contact with the originating lab to inquire about obtaining the immunogen peptide as your had requested. Unfortunately this peptide is not available. The antibody was raised against a synthetic pep...

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    Thank you for contacting Abcam.

    Have you already purchased ab36983 and are seeing bands at ˜180kDa in your western blot image? From the other Abreviews and publications that are associated with ab36983 (I have attached the Abreview western bl...

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    Thank you for contacting us.

    The product ab36983 was created from an immunogen at the C terminal regionf Human Niemann Pick C1 so that would not be appropriate for your needs. Of our other products, only ab116038 was crerated from and immuno...

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"