ABE2879M

Anti-Dyskerin (Sheep)

from sheep, purified by affinity chromatography

• Synonym(s): 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
• UNSPSC Code: 12352203
• eCl@ss: 32160702
• NACRES: NA.43

Properties

• biological source: sheep
• Quality Level: 100
• antibody form: affinity isolated antibody
• antibody product type: primary antibodies
• clone: polyclonal
• purified by: affinity chromatography
• species reactivity: human
• packaging: antibody small pack of 25 μg
• technique(s): ChIP: suitable (ChIP-seq); immunoprecipitation (IP): suitable; western blot: suitable
• isotype: IgG
• NCBI accession no.: NP_001354
• UniProt accession no.: O60832
• target post-translational modification: unmodified
• Gene Information: human ... DKC1(1736)

Description

General description

H/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).

Specificity

This sheep polyclonal antibody detects Dyskerin in human cells. It targets a 16 amino acid epitope within the C-terminal half of human Dyskerin.

Immunogen

Diphtheria Toxoid Carrier Protein-conugated linear peptide corresponding to 16 amino acids from the C-terminal half of human Dyskerin.

Epitope: C-terminal half

Application

Anti-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.

Research Category
Epigenetics & Nuclear Function

Western 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).

Quality

Evaluated by Western Blotting in HeLa cell Lysate.

Western Blotting Analysis: 2 µg/mL of this antibody detected Dyskerin in HeLa cell Lysate.

Target description

~58 kDa observed; 57.67 kDa calculated. Uncharacterized bands may be observed in some lysate(s).

Physical form

Affinity Purified

Purified sheep polyclonal antibody in buffer containing 20 mM HEPES-KOH (pH 7.9) 200 mM KCl 1 mM EDTA with 0.02% sodium azide.

Storage and Stability

Stable for 1 year at 2-8°C from date of receipt.

Other Notes

Concentration: Please refer to lot specific datasheet.

Disclaimer

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.

Safety Information

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: nwg
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable