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
9007-83-4
Specifications
ELISA (order BSA/sodium azide-free format for coating), Immunohistochemistry (FFPE) : 1-2 µg/mL for 30 min at RT
UniProt
P11532
Host
Mouse
Reactivity
Human
Immunogen
Amino acids 114-263 from the human protein were used as the immunogen for the Dystrophin antibody.
Clonality
Monoclonal
Isotype
IgG1 k
Clone
DMD/3243
Applications
ELISA, IHC-P
Purity
Protein G affinity chromatography
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.
Storage Conditions
Store the Dystrophin antibody at 2-8°C (with azide) or aliquot and store at -20°C or colder (without azide) .
Formulation
0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced) and 0.05% sodium azide
Location
Cell surface, cytoplasmic
Image Legend
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