DKC1 Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF,Flow-Cyt,ICC/IF | Bioss
Rrmab?兔单抗
货号：bsm-61870R
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概述
产品编号
bsm-61870R
产品类型
重组兔单抗
英文名称
DKC1 Recombinant Rabbit mAb
中文名称
核仁蛋白NAP57重组兔单抗
英文别名
CBF5; CHINE1; DKC; DKCX; NAP57; NOLA4; XAP101; Dkc1x; Dkc1y; DKC1_HUMAN; DKC1; CBF5 homolog; Dyskerin; Nopp140-associated protein of 57 kDa; Nucleolar protein NAP57; Nucleolar protein family A member 4; snoRNP protein DKC1; 5.4.99.-; DKC1_MOUSE; DKC1_RAT;
抗体来源
Rabbit
免疫原
A synthesized peptide derived from human Dyskerin: 350-400/514
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
克隆号
4A9
理论分子量
58 kDa
检测分子量
60 kDa
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
研究领域
SWISS
Gene ID
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. 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.
数据库链接
背景资料
Isoform 1: Catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. Isoform 3: Promotes cell to cell and cell to substratum adhesion, increases the cell proliferation rate and leads to cytokeratin hyper-expression.
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Human, Mouse | Rat | 1:500-2000 |
| IHC-P | Human, Mouse, Rat | 1:100-500 | |
| IHC-F | Human, Mouse, Rat | 1:100-500 | |
| IF | Human, Mouse, Rat | 1:100-500 | |
| Flow-Cyt | Human | Mouse, Rat | 1:50-100 |
| ICC/IF | Human, Mouse, Rat | 1:50-200 |
交叉反应
交叉反应: Human, Mouse, Rat
相关产品
暂无相关产品
靶标
基因名
DKC1
蛋白名
H/ACA ribonucleoprotein complex subunit DKC1
亚细胞定位
Cytoplasm and Nucleus, nucleolus. Nucleus, Cajal body. Also localized to Cajal bodies.
组织特异性
Ubiquitously expressed.
疾病
Defects in DKC1 are a cause of dyskeratosis congenita X-linked recessive (XDKC) [MIM:305000]. XDKC is a rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.
Defects in DKC1 are the cause of Hoyeraal-Hreidarsson syndrome (HHS) [MIM:300240]. HHS is a multisystem disorder affecting males and is characterized by aplastic anemia, immunodeficiency, microcephaly, cerebellar hypoplasia, and growth retardation.
相似性
Belongs to the pseudouridine synthase TruB family.
Contains 1 PUA domain.
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.
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