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重要文件

SAB4200763

Sigma-Aldrich

Anti-Dystrophin Antibody

enhanced validation

mouse monoclonal, MANDRA1

同義詞:

Anti-DMD

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.41

產品名稱

Anti-Dystrophin antibody, Mouse monoclonal, clone MANDRA1, purified from hybridoma cell culture

生物源

mouse

品質等級

抗體表格

purified from hybridoma cell culture

抗體產品種類

primary antibodies

無性繁殖

MANDRA1, monoclonal

形狀

buffered aqueous solution

分子量

~427 kDa

物種活性

zebrafish, rat, mouse, human

加強驗證

independent
Learn more about Antibody Enhanced Validation

濃度

~1.0 mg/mL

技術

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

同型

IgG1

UniProt登錄號

運輸包裝

dry ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... DMD(1756)

一般說明

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.

特異性

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.

應用

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.

外觀

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

儲存和穩定性

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.

免責聲明

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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儲存類別代碼

10 - Combustible liquids

閃點(°F)

Not applicable

閃點(°C)

Not applicable


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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
Duchenne muscular dystrophy and dystrophin: pathogenesis and opportunities for treatment
Nowak KJ and Davies KE
EMBO Reports, 5(9), 872-876 (2004)
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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