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Key Documents

MAB1692

Sigma-Aldrich

Anti-Dystrophin Antibody, mid-rod, clone 6D3

culture supernatant, clone 6D3, Chemicon®

Synonym(s):

Anti-BMD, Anti-CMD3B, Anti-DXS142, Anti-DXS164, Anti-DXS206, Anti-DXS230, Anti-DXS239, Anti-DXS268, Anti-DXS269, Anti-DXS270, Anti-DXS272, Anti-MRX85

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About This Item

UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

antibody form

culture supernatant

antibody product type

primary antibodies

clone

6D3, monoclonal

species reactivity

mouse, canine, human, rabbit, rat

manufacturer/tradename

Chemicon®

technique(s)

immunohistochemistry: suitable
western blot: suitable

isotype

IgG2a

UniProt accession no.

shipped in

dry ice

target post-translational modification

unmodified

Gene Information

human ... DMD(1756)

Specificity

Mid rod domain (between amino acids 1181 and 1388) of human dystrophin. Also reacts with skeletal, cardiac and smooth muscle dystrophin from normal mouse, rat, rabbit and dog. Other animal species have not been tested. Reacts on blots with the brain isoform. No reactivity with mdx mouse tissue or DMD/BMD patients who have a gene deletion which removes the antibody binding site. Does not react with chicken dystrophin.

STAINING PATTERN:Light microscopy: continuous rim of labeling at the periphery of muscle fibers.

E.M. gold: close to the cytoplasmic face of the plasma membrane.

Western blotting: strong double bands at approximately 400 kD plus metabolites of lower molecular mass.

Immunogen

Bacterial fusion protein (Cell (1987) 51:919-928).
Epitope: mid-rod

Application

Immunohistochemistry: (fresh frozen, unfixed tissue only): use

undiluted - 1:20. Not recommended for use on paraffin embedded tissue.

EM Gold (Light fixation with 2% formaldehyde + 0.001% glutaraldehyde for 1 hour. 2.3M sucrose used as cryoprotectant.): use undiluted. 90 minute incubation at 25°C.

Western blotting: use 1:100-1:250.

Optimal working dilutions must be determined by the end user.

Protocol for Immunohistochemical use of MAB1692

1) Freeze muscle blocks in isopentane chilled in liquid nitrogen.

2) Cut 4 μm to 10 μm sections and air dry on slides coated with 0.5% gelatin containing 0.05% chrome alum.

3) Slides may be stored at -70 °C wrapped in cling film until required. If stored sections are used, allow sections to equilibrate to room temperature before unwrapping and proceeding.

4) Apply a 50 μL aliquot of primary antibody to sections (unfixed). Incubate for 1 hour at room temperature or 37°C.

5) Wash sections 3 x 10 minutes in phosphate buffered saline.

6) Apply a 50 μL aliquot of labeled second antibody. Incubate for 60 minutes at 25°C.

7) Wash sections 3 x 10 minutes in phosphate buffered saline.

8) Mount fluorescent sections in aqueous mounting media or visualize peroxidase label (DAB). Dehydrate, clean and mount peroxidase labeled sections for permanent preparations.
Research Category
Metabolism
Research Sub Category
Muscle Physiology
This Anti-Dystrophin Antibody, mid-rod, clone 6D3 is validated for use in WB, IH for the detection of Dystrophin.

Physical form

Culture supernatant, liquid in PBS with 1% BSA, containing 15 mM sodium azide.

Storage and Stability

Maintain at -20°C for up to one year in convenient undiluted aliquots. Avoid repeated freeze/thaw cycles.

Analysis Note

Control
POSITIVE CONTROL: Snap frozen normal human or rat striated muscle.

Legal Information

CHEMICON 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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Storage Class Code

12 - Non Combustible Liquids

WGK

WGK 2

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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S Masuda et al.
Acta physiologica (Oxford, England), 195(4), 483-494 (2008-12-02)
The dystrophin-glycoprotein complex (DGC) and focal adhesion complex (FAC) are transmembrane structures in muscle fibres that link the intracellular cytoskeleton to the extracellular matrix. DGC and FAC proteins are abundant in slow-type muscles, indicating the structural reinforcement which play a
Daria Wojtal et al.
American journal of human genetics, 98(1), 90-101 (2015-12-22)
Clustered regularly interspaced short palindromic repeat (CRISPR) has arisen as a frontrunner for efficient genome engineering. However, the potentially broad therapeutic implications are largely unexplored. Here, to investigate the therapeutic potential of CRISPR/Cas9 in a diverse set of genetic disorders
beta-Dystroglycan binds caveolin-1 in smooth muscle: a functional role in caveolae distribution and Ca2+ release.
Sharma, P; Ghavami, S; Stelmack, GL; McNeill, KD; Mutawe, MM; Klonisch, T; Unruh, H; Halayko, AJ
Journal of Cell Science null
John Cw Hildyard et al.
PLoS currents, 8 (2016-08-16)
Exon-skipping via synthetic antisense oligonucleotides represents one of the most promising potential therapies for Duchenne muscular dystrophy (DMD), yet this approach is highly sequence-specific and thus each oligonucleotide is of benefit to only a subset of patients. The discovery that
Brain Dp140 alters glutamatergic transmission and social behaviour in the mdx52 mouse model of Duchenne muscular dystrophy.
Hashimoto, et al.
Progress in Neurobiology, 216, 102288-102288 (2022)

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