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Merck
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主要文件

SAB4200611

Sigma-Aldrich

Anti-DKC1 antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody

别名:

Anti-CBF5, Anti-CBF5 homolog, Anti-DKC, Anti-H/ACA ribonucleoprotein complex subunit 4, Anti-NAP57, Anti-NOLA4, Anti-XAP101, Anti-cbf5p homolog, Anti-dyskeratosis congenita 1, Anti-dyskerin, Anti-nopp140-associated protein of 57 kDa, Anti-nucleolar protein family A member 4, Anti-snoRNP protein DKC1

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About This Item

UNSPSC代码:
12352203
NACRES:
NA.41

生物来源

rabbit

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous solution

分子量

antigen ~57 kDa

种属反应性

human, mouse

浓度

~1.0 mg/mL

技术

immunoblotting: 0.5-1.0 μg/mL using whole extracts of mouse Hepa1-6 cells or nuclear extract of HeLa cells.

UniProt登记号

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... DKC1(1736)

一般描述

Dyskerin is a nucleolar protein encoded by the DKC1 gene. This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. The DKC1 gene lies in a tail-to-tail orientation with the palmitoylated erythrocyte membrane protein gene and is transcribed in a telomere to centromere direction.

免疫原

peptide corresponding to the N-terminal region of human DKC1, conjugated to KLH. The corresponding sequence is identical in monkey, bovine, pig and dog and differs by a single amino acid in mouse and rat.

应用

Anti-DKC1 antibody has been used in immunoblotting.

生化/生理作用

Dyskerin and the three NOLA proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Mutations in the DKC1 gene cause X-linked dyskeratosis congenita, aplastic anemia and progressive bone marrow failure in most cases. It also leads to a disease resulting in reticulate skin pigmentation, mucosal leukoplakia, nail dystrophy. It also causes Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita. The overexpression of dyskerin has been observed in many cancers including neuroblastoma, colorectal cancer, lymphoma, breast cancer, melanoma, prostate cancer, ovarian carcinoma and hepatocellular carcinoma.

外形

a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable


历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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Mouse dyskerin mutations affect accumulation of telomerase RNA and small nucleolar RNA, telomerase activity, and ribosomal RNA processing
Mochizuki Y, et al.
Proceedings of the National Academy of Sciences of the USA, 101(29), 10756-10761 (2004)
An enhanced H/ACA RNP assembly mechanism for human telomerase RNA
Egan E D and Collins K
Molecular and Cellular Biology, 32(13), 2428-2439 (2012)
Telomerase reverse-transcriptase homozygous mutations in autosomal recessive dyskeratosis congenita and Hoyeraal-Hreidarsson syndrome
Marrone A, et al.
Blood, 110(13), 4198-4205 (2007)
Dyskerin overexpression in human hepatocellular carcinoma is associated with advanced clinical stage and poor patient prognosis
Liu B, et al.
PLoS ONE, 7(8), e43147-e43147 (2012)
Brendan J Quinn et al.
Proceedings of the National Academy of Sciences of the United States of America, 106(47), 19842-19847 (2009-11-10)
As mediators of innate immunity, neutrophils respond to chemoattractants by adopting a highly polarized morphology. Efficient chemotaxis requires the formation of one prominent pseudopod at the cell front characterized by actin polymerization, while local inhibition suppresses the formation of rear

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