| 货号 | 53234S |
| 同种亚型 | Rabbit IgG |
| 反应种属 | Human\Mouse\Moneky |
| 来源宿主 | Rabbit IgG |
| 应用 | WB |
| 目标/特异性 | Dyskerin (D6N4K) Rabbit mAb recognizes endogenous levels of total dyskerin protein. |
| 使用方法 | W |
| 供应商 | CST |
| 灵敏度 | Endogenous |
| 背景 | 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). |
| 存放说明 | -20C |
| 计算分子量 | 60 |
| 参考文献 | 1 . Lafontaine, D.L. et al. (1998) Genes Dev 12, 527-37. 2 . Henras, A. et al. (1998) EMBO J 17, 7078-90. 3 . Watkins, N.J. et al. (1998) RNA 4, 1549-68. 4 . Mitchell, J.R. et al. (1999) Nature 402, 551-5. 5 . Heiss, N.S. et al. (1998) Nat Genet 19, 32-8. 6 . Knight, S.W. et al. (1999) Am J Hum Genet 65, 50-8. 7 . Yoon, A. et al. (2006) Science 312, 902-6. |
Western blot analysis of extracts from various cell lines using Dyskerin (D6N4K) Rabbit mAb. |
