Anti-Dyskerin (Rabbit)

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 rabbit polyclonal antibody detects Dyskerin in human cells. It targets a 16 amino acid epitope within the C-terminal half of the Dyskerin protein. For immunoprecipitaion the use of HEPES or Tris bufer with KCl is highly recommended.
• Immunogen: Epitope: unknown
• Application: Anti-Dyskerin (Rabbit), Cat. No. ABE2587, is a highly specific rabbit polyclonal antibody that targets H/ACA ribonucleoprotein complex subunit 4 and has been tested for use in Immunofluorescence, Immunoprecipitation, and in Western Blotting.
• Quality: Evaluated by Western Blotting in SW480 cell lysate.Western Blotting Analysis: A 1:500 dilution of this antibody detected Dyskerin in 10 µg of SW480 cell lysate.
• Target description: ~58 kDa observed; 57.67 kDa calculated. Uncharacterized bands may be observed in some lysate(s).
• Physical form: Affinity Purified
• 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.