DMD Antibody / 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.
Product Specifications
CAS Number
9007-83-4
Specifications
Immunofluorescence: 1-2 µg/mL, Western blot: 1-2 µg/mL, Immunohistochemistry (FFPE) : 1-2 µg/mL
UniProt
P11532
Host
Mouse
Reactivity
Human
Immunogen
A portion of amino acids 1700-2300 from the human Dystrophin was used as the immunogen for the DMD antibody.
Clonality
Monoclonal
Isotype
IgG2a κ
Clone
DMD/6270
Applications
IF, WB, 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 DMD antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
Limitations
This DMD antibody is available for research use only.
Storage Conditions
Aliquot the DMD antibody and store frozen at -20°C or colder. Avoid repeated freeze-thaw cycles.
Product Datasheet
https://www.nsjbio.com/tds-pdf/dmd-antibody-dystrophin-dmd6270-v4009
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 DMD antibody should be determined by the researcher.
Location
Cell surface, cytoplasm
Image Legend
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