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SAB4200763

Sigma-Aldrich

Anti-Dystrophin antibody, Mouse monoclonal

enhanced validation

clone MANDRA1, purified from hybridoma cell culture

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Synonym(s):
Anti-DMD
UNSPSC Code:
12352203
NACRES:
NA.41

biological source

mouse

Quality Level

200

antibody form

purified from hybridoma cell culture

antibody product type

primary antibodies

clone

MANDRA1, monoclonal

form

buffered aqueous solution

mol wt

~427 kDa

species reactivity

zebrafish, rat, mouse, human

enhanced validation

independent
Learn more about Antibody Enhanced Validation

concentration

~1.0 mg/mL

technique(s)

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

isotype

IgG1

UniProt accession no.

P11532

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... DMD(1756)

Related Categories

General description

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.

Specificity

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.

Application

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

Biochem/physiol Actions

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.

Physical form

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

Storage and Stability

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.

Disclaimer

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.

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Information

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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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