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.
Formulation
1 mg/ml in 1X PBS; BSA free, sodium azide free
Host
Mouse
Immunogen Region
Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
Isotype
Mouse IgG2b, kappa
Species Reactivity
Human
Note
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.
Uniprot
P11532
Format
Purified
Purity
Protein G affinity chromatography
Storage
Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Applications
ELISA, IHC-P
Description
ELISA (order BSA/sodium azide-free format for coating),Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT