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安全信息

HPA018803

Sigma-Aldrich

Anti-DES antibody produced in rabbit

enhanced validation

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

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别名:
Anti-Desmin
UNSPSC代码:
12352203
人类蛋白质图谱编号:
NACRES:
NA.41

生物来源

rabbit

质量水平

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

产品线

Prestige Antibodies® Powered by Atlas Antibodies

形式

buffered aqueous glycerol solution

种属反应性

human

增强验证

orthogonal RNAseq
Learn more about Antibody Enhanced Validation

技术

immunoblotting: 0.04-0.4 μg/mL
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:500-1:1000

免疫原序列

KLLEGEESRINLPIQTYSALNFRETSPEQRGSEVHTKKTVMIKTIETRDGEVVSEATQQQHEVL

UniProt登记号

运输

wet ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... DES(1674)

一般描述

The gene desmin (DES) is mapped to human chromosome 2q35. It is a member of the intermediate filament protein gene family. DES is strongly expressed in skeletal, cardiac and smooth muscle cells.

免疫原

Desmin recombinant protein epitope signature tag (PrEST)

应用

All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.

The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set 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.

生化/生理作用

Desmin (DES) is an important component of the extra-sarcomeric cytoskeleton. Mutation in DES is associated with cardiac phenotype characterized with right ventricular predominance. Mutations in DES are also associated with myofibrillar myopathies. Missense mutation of DES is the cause of idiopathic dilated cardiomyopathy. Mutation in DES, which causes disruption of DES-lamin-B interaction in the skeletal muscle, causes limb girdle muscular dystrophy phenotype. DES also interacts with myotubularin (MTM1). The complex is crucial for proper intermediate filament assembly and architecture in muscle cells. DES also interacts with α-actinin and actin (Z-disk proteins), nebulin, MLH1 (mismatch repair protein), myospryn, desmoplakin and dysferlin.

特点和优势

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.

联系

Corresponding Antigen APREST74605

外形

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

法律信息

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

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.

WGK

WGK 1

闪点(°F)

Not applicable

闪点(°C)

Not applicable

个人防护装备

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

法规信息

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D Li et al.
Circulation, 100(5), 461-464 (1999-08-03)
Idiopathic dilated cardiomyopathy, of which approximately 20% of cases are familial (FDCM), is a primary myocardial disorder characterized by ventricular dilatation and impaired systolic function. It is a common cause of heart failure and the need for cardiac transplantation. Although
Nilgun Cetin et al.
Journal of medical genetics, 50(7), 437-443 (2013-05-21)
Autosomal recessive limb girdle muscular dystrophy (LGMD2) is a heterogeneous group of myopathies characterised by progressive muscle weakness involving proximal muscles of the shoulder and pelvic girdles including at least 17 different genetic entities. Additional loci have yet to be
J Peter van Tintelen et al.
Heart rhythm, 6(11), 1574-1583 (2009-11-03)
Desmin-related myopathy is a clinically heterogenous group of disorders encompassing myopathies, cardiomyopathies, conduction disease, and combinations of these disorders. Mutations in the gene encoding desmin (DES), a major intermediate filament protein, can underlie this phenotype. The purpose of this study
Karim Hnia et al.
The Journal of clinical investigation, 121(1), 70-85 (2010-12-08)
Muscle contraction relies on a highly organized intracellular network of membrane organelles and cytoskeleton proteins. Among the latter are the intermediate filaments (IFs), a large family of proteins mutated in more than 30 human diseases. For example, mutations in the
Clifford J Whatcott et al.
PloS one, 12(8), e0183871-e0183871 (2017-08-26)
ROCK, or Rho-associated coiled coil-containing protein kinase, is a member of the AGC kinase family and has been shown to play a role in cell migration, ECM synthesis, stress-fiber assembly, and cell contraction. Increased ROCK expression has been reported in

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