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ABE2879 Anti-Dyskerin (Sheep)

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ABE2879
100 μg  
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      Replacement Information
      Description
      Catalogue NumberABE2879
      DescriptionAnti-Dyskerin (Sheep)
      Alternate Names
      • H/ACA ribonucleoprotein complex subunit 4
      • CBF5 homolog
      • Nopp140-associated protein of 57 kDa
      • Nucleolar protein NAP57
      • Nucleolar protein family A member 4
      • snoRNP protein DKC1
      Background InformationH/ACA ribonucleoprotein complex subunit 4 (UniProt: O60832; also known as CBF5 homolog, Dyskerin, Nopp140-associated protein of 57 kDa, Nucleolar protein NAP57, Nucleolar protein family A member 4, snoRNP protein DKC1) is encoded by the DKC1 (also known as NOLA4) gene (Gene ID: 1736) in human. Dyskerin is a highly conserved nucleolar protein that is involved with multiple cellular functions, such as protein expression, growth, proliferation, and nucleo-cytoplasmic shuttling. It helps in the stabilization of the telomerase RNA component, which allows for the proper functioning of telomerase enzymatic complex. It also modifies specific uridine residues of ribosomal RNA by converting them to pseudouridine, thus allowing proper ribosome processing and function. Point mutations in the DKC1 gene result in a rare inherited syndrome called X-linked dyskeratosis congenita, which is leads to early mortality due to progressive bone marrow failure and is characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. (Ref.: Montanaro, L (2010). J. Pathol. 222(4): 345-9).
      References
      Product Information
      FormatAffinity Purified
      PresentationPurified sheep polyclonal antibody in buffer containing 20 mM HEPES-KOH (pH 7.9) 200 mM KCl 1 mM EDTA with 0.02% sodium azide.
      Quality LevelMQ100
      Applications
      ApplicationAnti-Dyskerin (Sheep), Cat. No. ABE2879, is a highly specific sheep polyclonal antibody that targets H/ACA ribonucleoprotein complex subunit 4 and has been tested in Chromatin Immunoprecipitation (ChIP), Immunoprecipitation, and Western Blotting.
      Key Applications
      • ChIP-seq
      • Immunoprecipitation
      • Western Blotting
      Application NotesWestern Blotting Analysis: A representative lot detected Dyskerin in 293T cells. The signal was depleted upon treatment with siRNA against Dyskerin (Stern, J.L., et. al. (2012). Mol Cell Biol. 32(13):2384-95).

      Chromatin Immunoprecipitation (ChIP) Analysis: A representative lot detected Dyskerin associated to telomeres in telomerase-overexpressing HEK293 cells (Stern, J.L., et. al. (2012). Mol Cell Biol. 32(13):2384-95).

      Immunoprecipitation Analysis: A representative lot recoverd Dyskerin telomerase-overexpressing 293T cells. (Stern, J.L., et. al. (2012). Mol Cell Biol. 32(13):2384-95).

      Western Blotting Analysis: A representative lot detected Dyskerin in HEK293T cells transfected with a dyskerin expression plasmid (Courtesy of the laboratories of Dr. Hilda Pickett and Dr. Tracy Bryan, Children s Medical Research Institute, Australia).
      Biological Information
      ImmunogenDiphtheria Toxoid Carrier Protein-conugated linear peptide corresponding to 16 amino acids from the C-terminal half of human Dyskerin.
      EpitopeC-terminal half
      ConcentrationPlease refer to lot specific datasheet.
      HostSheep
      SpecificityThis sheep polyclonal antibody detects Dyskerin in human cells. It targets a 16 amino acid epitope within the C-terminal half of human Dyskerin.
      IsotypeIgG
      Species Reactivity
      • Human
      Species Reactivity NoteHuman.
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Gene Symbol
      • DKC1
      • NOLA4
      Purification MethodAffinity Purified
      UniProt Number
      Molecular Weight~58 kDa observed; 57.67 kDa calculated. Uncharacterized bands may be observed in some lysate(s).
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blotting in HeLa cell Lysate.

      Western Blotting Analysis: 2 µg/mL of this antibody detected Dyskerin in HeLa cell Lysate.
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 μg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Référence GTIN
      ABE2879 04054839339660

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