Filter:

WB

Supporting Data

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 mAb 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).

Database Links

UniProt ID: O60832

Entrez-Gene Id: 1736

Limited Uses

Except as otherwise expressly agreed in a writing signed by a legally authorized representative of CST, the following terms apply to Products provided by CST, its affiliates or its distributors. Any Customer's terms and conditions that are in addition to, or different from, those contained herein, unless separately accepted in writing by a legally authorized representative of CST, are rejected and are of no force or effect.

Products are labeled with For Research Use Only or a similar labeling statement and have not been approved, cleared, or licensed by the FDA or other regulatory foreign or domestic entity, for any purpose. Customer shall not use any Product for any diagnostic or therapeutic purpose, or otherwise in any manner that conflicts with its labeling statement. Products sold or licensed by CST are provided for Customer as the end-user and solely for research and development uses. Any use of Product for diagnostic, prophylactic or therapeutic purposes, or any purchase of Product for resale (alone or as a component) or other commercial purpose, requires a separate license from CST. Customer shall (a) not sell, license, loan, donate or otherwise transfer or make available any Product to any third party, whether alone or in combination with other materials, or use the Products to manufacture any commercial products, (b) not copy, modify, reverse engineer, decompile, disassemble or otherwise attempt to discover the underlying structure or technology of the Products, or use the Products for the purpose of developing any products or services that would compete with CST products or services, (c) not alter or remove from the Products any trademarks, trade names, logos, patent or copyright notices or markings, (d) use the Products solely in accordance with CST Product Terms of Sale and any applicable documentation, and (e) comply with any license, terms of service or similar agreement with respect to any third party products or services used by Customer in connection with the Products.

For Research Use Only. Not For Use In Diagnostic Procedures.

Cell Signaling Technology is a trademark of Cell Signaling Technology, Inc.

All other trademarks are the property of their respective owners. Visit our Trademark Information page.

Dyskerin (D6N4K) Rabbit mAb #53234