- Tel: 858.663.9055
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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
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Dystrophin Polyclonal Antibody recognizes dystrophin, a large cytoskeletal protein encoded by the DMD gene that plays an essential role in maintaining muscle fiber integrity. Dystrophin is a central component of the dystrophin-associated glycoprotein complex (DGC), which mechanically connects the intracellular actin cytoskeleton to the extracellular matrix through the muscle cell membrane. This connection helps stabilize the sarcolemma during repeated cycles of muscle contraction and relaxation.
Full-length dystrophin, commonly designated Dp427, has a molecular weight of approximately 427 kDa and contains several functionally distinct regions. Its N-terminal actin-binding domain interacts with filamentous actin, while a central rod domain composed of spectrin-like repeats provides structural flexibility. Cysteine-rich and C-terminal regions mediate interactions with components of the dystrophin-associated protein complex, including beta-dystroglycan, syntrophins, and dystrobrevins. Together, these interactions help distribute mechanical forces across the muscle membrane.
Dystrophin is highly expressed in skeletal and cardiac muscle, where it is concentrated along the sarcolemma. The DMD gene also produces shorter protein isoforms through alternative promoters and transcript processing. These isoforms exhibit tissue-specific expression patterns and contribute to dystrophin-related functions outside skeletal muscle, including roles in the nervous system. The existence of multiple dystrophin isoforms is important when interpreting antibody reactivity and protein expression in different tissues.
Pathogenic variants in the DMD gene cause Duchenne muscular dystrophy and Becker muscular dystrophy, two X-linked disorders characterized by progressive muscle weakness and degeneration. Duchenne muscular dystrophy typically results from variants that eliminate functional dystrophin expression, whereas Becker muscular dystrophy is generally associated with the production of reduced amounts or structurally altered dystrophin. Loss of dystrophin destabilizes the sarcolemma, increasing susceptibility to contraction-induced injury and contributing to inflammation, muscle fiber degeneration, and progressive replacement of muscle tissue by fibrotic and fatty tissue.
Dystrophin research also encompasses cardiac dysfunction, muscle regeneration, and therapeutic approaches designed to restore dystrophin expression. These include exon-skipping strategies, gene replacement approaches, and interventions intended to improve muscle membrane stability. Antibody-based methods support investigations of dystrophin expression, molecular weight, and tissue distribution in experimental models.
A Dystrophin Polyclonal Antibody from NSJ Bioreagents supports research investigating DMD protein expression, dystrophin-associated complexes, skeletal and cardiac muscle biology, and molecular mechanisms underlying muscular dystrophy.
This DMD antibody can be compared with our Dystrophin Antibody (clone DMD/3241) for analysis of dystrophin expression across muscle structure and neuromuscular disease studies.
The stated application concentrations are suggested starting amounts. Titration of the Dystrophin Polyclonal Antibody may be required due to differences in protocols and secondary/substrate sensitivity.
Human partial recombinant protein (AA 3076-3404) was used as the immunogen for this Dystrophin Polyclonal Antibody.
After reconstitution, the Dystrophin Polyclonal Antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
DMD antibody, Dystrophin Protein antibody, Dystrophin Muscle Protein antibody, Dystrophin Dp427 antibody
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