Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)(DMD/3241) , 1mg / mL
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. Primary antibodies are available purified , or with a selection of fluorescent CF® Dyes and other labels. CF® Dyes offer exceptional brightness and photostability. Note: Conjugates of blue fluorescent dyes like CF®405S and CF®405M are not recommended for detecting low abundance targets , because blue dyes have lower fluorescence and can give higher non-specific background than other dye colors.