DMD
Reactivity: Human
IHC, ELISA
Host: Rabbit
Polyclonal
unconjugated
Application 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.
Restrictions
For Research Use only
Format
Liquid
Concentration
1 mg/mL
Buffer
1 mg/mL in 1X PBS, BSA free, sodium azide free
Preservative
Azide free
Storage
4 °C,-20 °C
Storage Comment
Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Target
Dystrophin (DMD)
Alternative Name
Dystrophin
Background
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.