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Dystrophin Antibody / DMD

Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.

Product Specifications

UniProt

P11532

Reactivity

Human

Immunogen

Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.

Clonality

Monoclonal

Clone

DMD/3241

Conjugation

Unconjugated

Field of Research

Neuroscience; Musculoskeletal & Connective Tissue Research

Purification

Protein G affinity chromatography

Dilution

The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

Storage Conditions

Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Notes

For research use only.

Applications Notes

Optimal dilution of the Dystrophin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

Tested Applications

IHC-P

Host or Source

Mouse

Preservative

Prediluted in 1X PBS, 0.1 mg/ml rAlbumin, 0.05% sodium azide; For IHC use only

Isotype

Mouse IgG1, kappa

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