Overview

  • Product name

    Anti-DKC1/Dyskerin antibody [EPR10399]
    See all DKC1/Dyskerin primary antibodies
  • Description

    Rabbit monoclonal [EPR10399] to DKC1/Dyskerin
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, ICC/IF, IHC-Pmore details
    Unsuitable for: Flow Cyt or IP
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human DKC1/Dyskerin aa 350-450. The exact sequence is proprietary.
    Database link: O60832

  • Positive control

    • Recombinant Human DKC1/Dyskerin protein (ab152334) can be used as a positive control in WB. SW480 cell lysate, MCF-7 cell lysate, HeLa cell lysate, paraffin-embedded Human melanoma tissue, paraffin-embedded Human placenta tissue.
  • General notes

     This product was previously labelled as DKC1

     

    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.

Properties

Applications

Our Abpromise guarantee covers the use of ab156877 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
WB 1/1000 - 1/10000. Predicted molecular weight: 58 kDa.
ICC/IF 1/100 - 1/250.
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol.
  • Application notes
    Is unsuitable for Flow Cyt or IP.
  • Target

    • Function

      Isoform 1: Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.
      Isoform 3: Promotes cell to cell and cell to substratum adhesion, increases the cell proliferation rate and leads to cytokeratin hyper-expression (when overexpressed in HeLa cells).
    • Tissue specificity

      Ubiquitously expressed.
    • Involvement in disease

      Defects in DKC1 are a cause of dyskeratosis congenita X-linked recessive (XDKC) [MIM:305000]. XDKC is a rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.
      Defects in DKC1 are the cause of Hoyeraal-Hreidarsson syndrome (HHS) [MIM:300240]. HHS is a multisystem disorder affecting males and is characterized by aplastic anemia, immunodeficiency, microcephaly, cerebellar hypoplasia, and growth retardation.
    • Sequence similarities

      Belongs to the pseudouridine synthase TruB family.
      Contains 1 PUA domain.
    • Cellular localization

      Cytoplasm and Nucleus > nucleolus. Nucleus > Cajal body. Also localized to Cajal bodies.
    • Information by UniProt
    • Database links

    • Alternative names

      • CBF5 antibody
      • CBF5 homolog antibody
      • Cbf5p homolog antibody
      • DKC 1 antibody
      • DKC antibody
      • Dkc1 antibody
      • DKC1_HUMAN antibody
      • DKCX antibody
      • Dyskeratosis congenita 1 antibody
      • Dyskeratosis congenita 1 dyskerin antibody
      • Dyskerin antibody
      • H/ACA ribonucleoprotein complex subunit 4 antibody
      • NAP 57 antibody
      • NAP57 antibody
      • NOLA 4 antibody
      • NOLA4 antibody
      • Nopp140 associated protein of 57 kDa antibody
      • Nopp140-associated protein of 57 kDa antibody
      • Nucleolar protein family A member 4 antibody
      • Nucleolar protein NAP57 antibody
      • snoRNP protein DKC1 antibody
      • XAP 101 antibody
      • XAP101 antibody
      see all

    Images

    • All lanes : Anti-DKC1/Dyskerin antibody [EPR10399] (ab156877) at 1/1000 dilution

      Lane 1 : SW480 cell lysate
      Lane 2 : HeLa cell lysate
      Lane 3 : MCF-7 cell lysate

      Lysates/proteins at 10 µg per lane.

      Developed using the ECL technique.

      Predicted band size: 58 kDa

    • Immunohistochemical analysis of paraffin embedded Human melanoma tissue labeling DKC1/Dyskerin with ab156877 antibody at a dilution of 1/50.

    • Immunohistochemical analysis of paraffin embedded Human placenta tissue labeling DKC1/Dyskerin with ab156877 antibody at a dilution of 1/50.

    • Immunofluorescence analysis of HeLa cells labeling DKC1/Dyskerin with ab156877 antibody at a dilution of 1/100.

    References

    This product has been referenced in:

    • Quan Wang J  et al. Discovery of new chromen-4-one derivatives as telomerase inhibitors through regulating expression of dyskerin. J Enzyme Inhib Med Chem 33:1199-1211 (2018). Read more (PubMed: 30132373) »
    See 1 Publication for this product

    Customer reviews and Q&As

    Application
    Western blot
    Sample
    Human Cell lysate - whole cell (HES2 human embryonic stem cell, 293T, HeLa)
    Gel Running Conditions
    Reduced Denaturing
    Loading amount
    50 µg
    Specification
    HES2 human embryonic stem cell, 293T, HeLa
    Blocking step
    Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: RT°C

    Jianlong Wang

    Verified customer

    Submitted Dec 22 2017

    Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
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