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この商品について
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IF, IHC
Citations:
10
biological source
rabbit
Quality Level
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
enhanced validation
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
technique(s)
immunoblotting: 0.04-0.4 μg/mL, immunofluorescence: 0.25-2 μg/mL, immunohistochemistry: 1:500-1:1000
immunogen sequence
KLLEGEESRINLPIQTYSALNFRETSPEQRGSEVHTKKTVMIKTIETRDGEVVSEATQQQHEVL
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... DES(1674)
General description
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.
Immunogen
デスミンのPrEST(protein epitope signature tag)抗原リコンビナントタンパク質。
Application
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
Biochem/physiol Actions
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.
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:
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.
Physical form
PBS溶液(pH 7.2, 40%グリセロールおよび0.02%アジ化ナトリウム含有)。
Other Notes
Corresponding Antigen APREST74605
Legal Information
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
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.
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保管分類
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)
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
HPA018803-25UL: + HPA018803-100UL:
jan
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
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
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