Ricombinante Anti-Dystrophin Anticorpo [DMD/8773R] (A314899)

Anti-Dystrophin Antibody [DMD/8773R]

Recombinant rabbit monoclonal [DMD/8773R] antibody to Dystrophin.

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.

IF, WB, IHC-P

Human skeletal muscle and heart muscle tissues (IHC).

IF: 1-2 µg/ml, WB: 1-2 µg/ml, IHC: 1-2 µg/ml

Human

Recombinant fragment (around Amino Acids 1700-2300) of human DMD. The exact sequence is proprietary..

Rabbit

Monoclonal

DMD/8773R

kappa

Unconjugated

Protein A/G chromatography.

200 µg/ml

427 kDa

Liquid

Supplied in 10mM Phosphate Buffered Saline with 0.05% BSA and 0.05% Sodium Azide.

Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

BMD, CMD3B, DMD, DMD_HUMAN, Duchenne muscular dystrophy protein, Muscular dystrophy Duchenne and Becker types