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Documenti fondamentali

SAB4200763

Sigma-Aldrich

Anti-Dystrophin Antibody

enhanced validation

mouse monoclonal, MANDRA1

Sinonimo/i:

Anti-DMD

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Nome del prodotto

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

Origine biologica

mouse

Livello qualitativo

Forma dell’anticorpo

purified from hybridoma cell culture

Tipo di anticorpo

primary antibodies

Clone

MANDRA1, monoclonal

Stato

buffered aqueous solution

PM

~427 kDa

Reattività contro le specie

zebrafish, rat, mouse, human

Convalida avanzata

independent
Learn more about Antibody Enhanced Validation

Concentrazione

~1.0 mg/mL

tecniche

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

Isotipo

IgG1

N° accesso UniProt

Condizioni di spedizione

dry ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... DMD(1756)

Descrizione generale

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.

Specificità

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.

Applicazioni

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

Azioni biochim/fisiol

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.

Stato fisico

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

Stoccaggio e stabilità

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.

Esclusione di responsabilità

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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Raccomandato

Codice della classe di stoccaggio

10 - Combustible liquids

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable


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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)
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

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