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Merck
CN

SAB4200763

Sigma-Aldrich

Anti-Dystrophin antibody, Mouse monoclonal

enhanced validation

clone MANDRA1, purified from hybridoma cell culture

别名:

Anti-DMD

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About This Item

UNSPSC代码:
12352203
NACRES:
NA.41

生物来源

mouse

质量水平

抗体形式

purified from hybridoma cell culture

抗体产品类型

primary antibodies

克隆

MANDRA1, monoclonal

形式

buffered aqueous solution

分子量

~427 kDa

种属反应性

zebrafish, rat, mouse, human

增强验证

independent
Learn more about Antibody Enhanced Validation

浓度

~1.0 mg/mL

技术

immunoblotting: suitable
immunofluorescence: suitable
immunohistochemistry: 10-20 μg/mL using acetone fixed rat tongue frozen sections

同位素/亚型

IgG1

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 ... DMD(1756)

相关类别

一般描述

Anti-Dystrophin antibody, Mouse monoclonal (mouse IgG1 isotype) is derived from the MANDRA1 hybridoma produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice. Dystrophin is a rod-shaped cytoskeletal protein located to the periphery (plasma membrane) of normal striated muscle fibers.
Dystrophin is encoded by the gene mapped to human chromosome Xp21.2-p21.1.

特异性

Anti-Dystrophin antibody, Mouse monoclonal specifically recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human Dystrophin molecule (residues 3558-3684). The antibody reacts with Dystrophin from human, mouse, rat and zebrafish origin. The antibody is specific to dystrophin and does not react with -actinin or utrophin.

应用

Anti-Dystrophin antibody, Mouse monoclonal has been used in:
  • immunohistochemistry
  • immunoblotting
  • immunofluorescence
  • enzyme-linked immunosorbent assay (ELISA)

生化/生理作用

Dystrophin is absent, reduced or altered because of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or in its homologue in mouse. Severe DMD is associated with a marked Dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression.

外形

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

储存及稳定性

For continuous use, store at 2–8 °C for up to one month. For extended storage, freeze in working aliquots. Repeated freezing and thawing is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.

免责声明

Unless otherwise stated in our catalog, 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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闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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在文件库中查找您最近购买产品的文档。

访问文档库

Duchenne muscular dystrophy and dystrophin: pathogenesis and opportunities for treatment
Nowak KJ and Davies KE
EMBO Reports, 5(9), 872-876 (2004)
Davide Rovina et al.
Stem cell research, 40, 101544-101544 (2019-08-30)
Duchenne's muscular dystrophy (DMD) is a neuromuscular disorder affecting skeletal and cardiac muscle function, caused by mutations in the dystrophin (DMD) gene. Dermal fibroblasts, isolated from a DMD patient with a reported deletion of exons 51 to 53 in the
Analysis of dystrophin gene deletions by multiplex PCR in Moroccan patients
Sbiti A, et al.
BioMed Research International, 2(3), 158-160 (2002)

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