CST, 53234S, Dyskerin (D6N4K) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying dyskerin. Validated for Western Blotting. Available in 2 sizes. Highly specific and rigorously validated in-house, Dyskerin (D6N4K) Rabbit Monoclonal Antibody (CST #53234) is ready to ship. Product Usage Information 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 Available protocols: Western Blotting 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 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 Specification REACTIVITY: H M Mk SENSITIVITY: Endogenous MW (kDa): 60 Source/Isotype: Rabbit IgG