Key Product Details

Species Reactivity

Human

Applications

ELISA, Immunohistochemistry-Paraffin, Protein Array

Label

PE (Excitation = 488 nm, Emission = 575 nm)

Antibody Source

Monoclonal Mouse IgG1 kappa Clone # DMD/3244

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

View all Dystrophin Antibodies »

Product Specifications

Immunogen

A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary) (Uniprot: P11532)

Localization

Cell Surface and Cytoplasmic

Marker

Marker of Duchenne and Becker Muscular Dystrophy

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1 kappa

Applications for Dystrophin Antibody (DMD/3244) [PE]

Application
Recommended Usage

ELISA

Optimal dilutions of this antibody should be experimentally determined.

Immunohistochemistry-Paraffin

Optimal dilutions of this antibody should be experimentally determined.

Protein Array

Optimal dilutions of this antibody should be experimentally determined.
Application Notes
Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Purification

Protein A or G purified

Formulation

PBS

Preservative

0.05% Sodium Azide

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

Shipping

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

Stability & Storage

Store at 4C in the dark.

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/3244) [PE]

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/3244) [PE]

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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