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Anti-Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Monoclonal Antibody (Clone: DMD/3242)

Selected Variant

36-2166-20

Size: 20 µg

Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, throμgh 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 thoμght to influence calcium ion homeostasis and force transmission.

Product Specifications

Product Name Alternative

BMD; CMD3B; Duchenne muscular dystrophy (DMD) ; Dystrophin; Muscular dystrophy Duchenne and Becker types

Gene Name

DMD

Gene ID

1756

UniProt

P11532

Host

Mouse

Reactivity

Human

Immunogen

A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary)

Clonality

Monoclonal

Clone

DMD/3242

Applications

ELISA, IHC

Components

200 μg/mL of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/mL.

Storage Conditions

Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C. Antibody is stable for 24 months. Non-hazardous.

Applications Notes

ELISA (For coating, order Ab without BSA) ; , Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT), (Staining of formalin-fixed tissues requires heating tissue sections in 10mM Tris with 1mM EDTA, pH 9.0, for 45 min at 95&degC followed by cooling at RT for 20 minutes)

Isotype

Mouse IgG2b, kappa

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