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- Bioss
- bs-13007P
- 2025年10月16日
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500ug
| 产品编号 | bs-13007P |
| 英文名称 | DKC1 Antibody Blocking Peptide |
| 中文名称 | 核仁蛋白NAP57封闭多肽 |
| 英文别名 | CBF5; CBF5 homolog; Cbf5p homolog; DKC 1; DKC; Dkc1; DKC1_HUMAN; DKCX; Dyskeratosis congenita 1; Dyskeratosis congenita 1 dyskerin; Dyskerin; H/ACA ribonucleoprotein complex subunit 4; NAP 57; NAP57; NAP-57; NOLA 4; NOLA4; Nopp140 associated protein of 57 kDa; Nopp140-associated protein of 57 kDa; Nucleolar protein family A member 4; Nucleolar protein NAP57; snoRNP protein DKC1; XAP 101; XAP101. |
| 纯化方法 | HPLC |
| 研究领域 | Epigenetics and Nuclear Signaling > Chromatin Binding Proteins > DNA / RNA binding Epigenetics and Nuclear Signaling > Chromosome Structure > Telomeres Epigenetics and Nuclear Signaling > DNA / RNA > RNA Processing Epigenetics and Nuclear Signaling > DNA / RNA > Translation > Ribosome |
| 亚细胞定位 | Cytoplasm and Nucleus, nucleolus. Nucleus, Cajal body. Also localized to Cajal bodies. |
| 组织特异性 | Ubiquitously expressed. |
| 相似性 | Belongs to the pseudouridine synthase TruB family. Contains 1 PUA domain. |
| 功能 | Isoform 1: Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine (1). GAR1, NHP2 and dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies (2). The dyskerin gene maps to chromosome Xq28 (3). Missense mutations in the dyskerin gene interfere with normal nuclear localization of dyskerin and cause Dyskeratosis congenita (DKC) (4). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 (5,6). The NHP2 gene maps to chromosome 5q35.3 and encodes a 155-amino acid protein (2,7). |
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DKC1 Antibody Blocking Peptide(bs-13007P)-500ug
￥880
