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
A recombinant partial protein sequence (within amino acids 1700-2300) from the human protein was used as the immunogen for the Dystrophin antibody.
Clonality
Recombinant
Clone
DMD/8773R
Conjugation
Unconjugated
Field of Research
Neuroscience; Musculoskeletal & Connective Tissue Research
Purification
Protein A/G affinity
Dilution
Immunohistochemistry (FFPE) : 1-2ug/ml for 30 min at RT
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.
Tested Applications
IHC-P
Host or Source
Rabbit
Preservative
0.2 mg/ml in 1X PBS with 0.1 mg/ml rAlbumin, 0.05% sodium azide
Isotype
Rabbit IgG, kappa
Available Sizes
Frequently Asked Questions
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