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/3241) [Alexa Fluor® 405]

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COA

SDS

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Product Specific Notices for Dystrophin Antibody (DMD/3241) [Alexa Fluor® 405]

Alexa Fluor (R) products are provided under an intellectual property license from Life Technologies Corporation. The purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: (i) in manufacturing; (ii) to provide a service, information, or data in return for payment; (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5791 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@lifetech.com. This conjugate is made on demand. Actual recovery may vary from the stated volume of this product. The volume will be greater than or equal to the unit size stated on the datasheet.

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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Dystrophin Antibody (DMD/3241) [Alexa Fluor® 405]

Novus Biologicals, part of Bio-Techne | Catalog # NBP2-79910AF405

Key Product Details

Species Reactivity

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Product Summary for Dystrophin Antibody (DMD/3241) [Alexa Fluor® 405]

Immunogen

Localization

Marker

Clonality

Host

Isotype

Applications for Dystrophin Antibody (DMD/3241) [Alexa Fluor® 405]

ELISA

Immunohistochemistry

Immunohistochemistry-Paraffin

Protein Array

Formulation, Preparation, and Storage

Purification

Formulation

Preservative

Concentration

Shipping

Stability & Storage

Background: Dystrophin