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
CAS Number
9000-83-3
Specifications
Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 min at RT
UniProt
P11532
Host
Rabbit
Reactivity
Human
Immunogen
A recombinant partial protein sequence (within amino acids 1700-2300) from the human protein was used as the immunogen for the Dystrophin antibody.
Clonality
Recombinant Monoclonal
Isotype
IgG κ
Clone
DMD/8773R
Type
Recombinant
Applications
IHC-P
Purity
Protein A/G affinity
Format
Purified
Buffer
0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide
Reconstitution
Aliquot the Dystrophin antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Limitations
This Dystrophin antibody is available for research use only.
Storage Conditions
Aliquot the Dystrophin antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
Formulation
0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05% sodium azide
Applications Notes
Optimal dilution of the Dystrophin antibody should be determined by the researcher.
Location
Cell surface, Cytoplasm
Image Legend
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