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Dyskerin (D6N4K) Rabbit Monoclonal Antibody #53234

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  • WB

    Product Specifications

    REACTIVITY H M Mk
    SENSITIVITY Endogenous
    MW (kDa) 60
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    Species Cross-Reactivity Key:
    • H-Human 
    • M-Mouse 
    • Mk-Monkey 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    Protocol

    Specificity / Sensitivity

    Dyskerin (D6N4K) Rabbit Monoclonal Antibody recognizes endogenous levels of total dyskerin protein.

    Species Reactivity:

    Human, Mouse, Monkey

    Source / Purification

    Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Phe91 of human dyskerin protein.

    Background

    H box/ACA-motif small nucleolar RNAs (snoRNAs) guide snoRNA proteins (snoRNPs) to uridine residues on rRNA for the conversion to pseudouridine (1). These H/ACA snoRNPs consist of four highly conserved proteins including GAR1, NHP2, NOP10, and the catalytic component dyskerin (1-3). The core snoRNPs also bind to mammalian telomerase RNA, which contains a H/ACA-like motif in the 3’ domain. This binding results in the maintenance of telomerase levels and activity (4). Defects in the snoRNPs can lead to dyskeratosis congenita, a rare, x-linked disorder characterized by a failure of the bone marrow and an increased tumor risk (5,6). Mutations in the dyskerin gene can cause defects in translation of mRNAs containing internal ribosome entry sites (IRESs), which include mRNAs to tumor suppressors p27 and p53 and anti-apoptotic factors Bcl-xL and XIAP (7).

    Alternate Names

    CBF5; CBF5 homolog; cbf5p homolog; DKC; DKC1; DKCX; dyskeratosis congenita 1, dyskerin; Dyskerin; dyskerin pseudouridine synthase 1; FLJ97620; H/ACA ribonucleoprotein complex subunit 4; H/ACA ribonucleoprotein complex subunit DKC1; NAP57; NOLA4; Nopp140-associated protein of 57 kDa; Nucleolar protein family A member 4; Nucleolar protein NAP57; snoRNP protein DKC1; XAP101

    For Research Use Only. Not for Use in Diagnostic Procedures.
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