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/3245
Conjugation
Unconjugated
Field of Research
Neuroscience; Musculoskeletal & Connective Tissue Research
Purification
Protein G affinity chromatography
Dilution
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
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 IgG2a, kappa
Frequently Asked Questions
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