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

ELISA (order BSA-free format for coating), Immunohistochemistry (FFPE) : 1-2 µg/mL

UniProt

P11532

Host

Mouse

Reactivity

Human

Immunogen

A portion of amino acids 114-263 from the human protein was used as the immunogen for the DMD antibody.

Clonality

Monoclonal

Isotype

IgG2b κ

Clone

DMD/3676

Applications

ELISA, 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.

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

IHC staining of FFPE human skeletal muscle tissue with DMD antibody (clone DMD/3676) . HIER: boil tissue sections in pH 9 10mM Tris with 1mM EDTA for 20 min and allow to cool before testing.