Recombinant
RabMAb

Recombinant Anti-DKC1/Dyskerin antibody [EPR10398] (ab156006)

Overview

  • Product name

    Anti-DKC1/Dyskerin antibody [EPR10398]
    See all DKC1/Dyskerin primary antibodies
  • Description

    Rabbit monoclonal [EPR10398] to DKC1/Dyskerin
  • Host species

    Rabbit
  • Tested applications

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

    Reacts with: Mouse, Rat, Human
  • Immunogen

    Tissue, cells or virus within Human DKC1/Dyskerin aa 100-200. The exact sequence is proprietary.
    Database link: O60832

  • Positive control

    • SW480, MCF7, HeLa, and HepG2 whole cell lysate (ab7900), HeLa cells
  • 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 ab156006 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. Detects a band of approximately 58 kDa.
ICC/IF 1/100 - 1/250.
  • Application notes
    Is unsuitable for Flow Cyt,IHC-P 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 [EPR10398] (ab156006) at 1/1000 dilution

      Lane 1 : SW480 cell lysate
      Lane 2 : MCF7 cell lysate
      Lane 3 : HeLa cell lysate
      Lane 4 : HepG2 cell lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Observed band size: 58 kDa
      why is the actual band size different from the predicted?

    • Immunofluorescent analysis of HeLa cells labeling DKC1/Dyskerin with ab156006 at 1/100.

    References

    ab156006 has not yet been referenced specifically in any publications.

    Customer reviews and Q&As

    Application
    Western blot
    Sample
    Human Cell lysate - whole cell (HES2 human embryonic stem cells, 293T, HeLa)
    Gel Running Conditions
    Reduced Denaturing
    Loading amount
    50 µg
    Specification
    HES2 human embryonic stem cells, 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 Jan 03 2018

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