Dystrophin Antibody / DMD
CAT:
800-V7562IHC-7ML
Size:
7 mL
Price:
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- Availability: 24/48H Stock Items & 2 to 6 Weeks non Stock Items.
- Dry Ice Shipment: No
Dystrophin Antibody / DMD
- Description: 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.
- CAS Number: 9007-83-4
- 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: IHC-P
- Format: Purified
- Buffer: Prediluted in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide; *For IHC use only*
- 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.