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
Host
Mouse
Immunogen
Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
Clonality
Monoclonal
Isotype
IgG1 κ
Applications
ELISA, IHC-P
Format
Purified
Buffer
0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide
Reconstitution
Store the Dystrophin antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).
Limitations
This Dystrophin antibody is available for research use only.
CAS Number
9007-83-4