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Merck
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HPA000166

Anti-DKC1 antibody produced in rabbit

Ab1, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

别名:

Anti-CBF5 homolog antibody produced in rabbit, Anti-Dyskerin antibody produced in rabbit, Anti-H/ACA ribonucleoprotein complex subunit 4 antibody produced in rabbit, Anti-Nopp140-associated protein of 57 kDa antibody produced in rabbit, Anti-Nucleolar protein NAP57 antibody produced in rabbit, Anti-Nucleolar protein family A member 4 antibody produced in rabbit, Anti-snoRNP protein DKC1 antibody produced in rabbit

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关于此项目

UNSPSC Code:
12352203
NACRES:
NA.43
Human Protein Atlas Number:
HPA000166

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产品名称

Anti-DKC1 antibody produced in rabbit, Ab1, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

product line

Prestige Antibodies® Powered by Atlas Antibodies

form

buffered aqueous glycerol solution

species reactivity

human

technique(s)

immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:50-1:200

immunogen sequence

EAGTYIRTLCVHLGLLLGVGGQMQELRRVRSGVMSEKDHMVTMHDVLDAQWLYDNHKDESYLRRVVYPLEKLLTSHKRLVMKDSAVNAICYGAKIMLPGVLRYEDGIEVNQEIVVIT

UniProt accession no.

O60832

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Quality Level

100

Gene Information

human ... DKC1(1736)

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Physical form

Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide

Legal Information

Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany

Application

All Prestige Antibodies®Powered by Atlas Antibodies is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.

Biochem/physiol Actions

Mutation in the DKC1 (Dyskeratosis congenita 1) gene will lead to the X-linked form of the disease called as Dyskeratosis congenita (DKC), a rare inherited bone marrow-failure syndrome. DKC is characterised by multiple features including nail dystrophy, abnormal skin pigmentation and increased susceptibility to cancer. Altered DKC1 gene will cause depletion in bone marrow production which is one of the major causes for premature death. DKC is a nucleolar protein with multiple roles including centromere function, rRNA transcription and nucleocytoplasmic trafficking etc.

Disclaimer

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.

Features and Benefits

Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.

Every Prestige Antibody is tested in the following ways:
  • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
  • Protein array of 364 human recombinant protein fragments.

General description

Dyskeratosis congenita 1 (DKC1) gene encodes dyskerin, which is a 514-amino-acid protein. This gene consists of 15 exons comprising a region of 15kb, with the internal exons size range of 65-185bp.

Immunogen

H/ACA ribonucleoprotein complex subunit 4 recombinant protein epitope signature tag (PrEST)

Other Notes

Corresponding Antigen APREST74148

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存储类别

10 - Combustible liquids

wgk

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

法规信息

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历史批次信息供参考:

分析证书(COA)

Lot/Batch Number
A37307
R00143
A00878
R000143
A000878

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Josu de la Fuente et al.
Pediatric transplantation, 11(6), 584-594 (2007-08-01)
DC is a multisystem bone marrow failure syndrome exhibiting marked clinical and genetic heterogeneity. X-linked, autosomal dominant and autosomal recessive subtypes are recognized. The gene mutated in X-linked DC (DKC1) encodes a highly conserved nucleolar protein called dyskerin. Dyskerin associates
M Kirwan et al.
Clinical genetics, 73(2), 103-112 (2007-11-17)
Dyskeratosis congenita (DC) is an inherited syndrome exhibiting marked clinical and genetic heterogeneity. It is characterized by multiple features including mucocutaneous abnormalities, bone marrow failure and an increased predisposition to cancer. Three genetic subtypes are recognized: X-linked recessive DC bears
S W Knight et al.
American journal of human genetics, 65(1), 50-58 (1999-06-12)
Dyskeratosis congenita is a rare inherited bone marrow-failure syndrome characterized by abnormal skin pigmentation, nail dystrophy, and mucosal leukoplakia. More than 80% of patients develop bone-marrow failure, and this is the major cause of premature death. The X-linked form of

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