Key Product Details

Species Reactivity

Human, Canine

Applications

Immunocytochemistry/ Immunofluorescence, Immunohistochemistry-Paraffin, Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG2a Kappa Clone # DMD/6270

Format

Azide and BSA Free

Concentration

1 mg/ml

View all Dystrophin Antibodies »

Product Specifications

Immunogen

Recombinant fragment (around aa1700-2300) of human Dystrophin (exact sequence is proprietary).

Reactivity Notes

Does not react with mouse, xenopus or chicken

Localization

Cell surface. Cytoplasm.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG2a Kappa

Description

Positive Controls: Human skeletal muscle and heart muscle tissues (IHC).

Antibody with azide - store at 2 to 8 C. Antibody without azide - store at -20 to -80 C. Non-hazardous. No MSDS required.

Scientific Data Images for Dystrophin Antibody (DMD/6270) - Azide and BSA Free

Dystrophin Antibody (DMD/6270) - Azide and BSA Free

Dystrophin Antibody (DMD/6270)

Formalin-fixed, paraffin-embedded human heart muscle stained with Dystrophin antibody (DMD/6270). HIER: Tris/EDTA, pH9.0, 45min. Secondary: HRP-polymer, 30min. DAB, 5min.

Applications for Dystrophin Antibody (DMD/6270) - Azide and BSA Free

Application
Recommended Usage

Immunocytochemistry/ Immunofluorescence

1-2 ug/ml

Immunohistochemistry-Paraffin

1-2 ug/ml

Western Blot

1-2 ug/ml
Application Notes
Immunohistochemistry (Formalin-fixed): 1-2ug/ml for 30 minutes. at RT. Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95C followed by cooling at RT for 20 minutes.

Optimal dilution for a specific application should be determined.

Formulation, Preparation, and Storage

Purification

Protein A or G purified

Formulation

10mM PBS

Format

Azide and BSA Free

Preservative

No Preservative

Concentration

1 mg/ml

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at -20 to -70C. Avoid freeze-thaw cycles.

Background: Dystrophin

Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse.8 Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine.9 The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies. Monoclonal antibodies against defined regions10 of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.11

Alternate Names

BMDDXS272, CMD3B, DXS142, DXS164, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, dystrophin, dystrophin (muscular dystrophy, Duchenne and Becker types), includes DXS142

Gene Symbol

DMD

Additional Dystrophin Products

Product Documents for Dystrophin Antibody (DMD/6270) - Azide and BSA Free

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Download SDS

Product Specific Notices for Dystrophin Antibody (DMD/6270) - Azide and BSA Free

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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