Home >> Antibodies >> Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)

Mouse Monoclonal Antibody [Clone DMD/3677]

Printable

Key References

Bioinformatics

Catalog #

Size

20 ug

100 ug

Price (USD)

219.00

499.00

Formulation

Purified Ab with BSA and Azide at 200ug/ml

Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Performance Guarantee

Catalog No

Size

20 ug

100 ug

Formulation

Purified Ab with BSA and Azide at 200ug/ml

Purified Ab WITHOUT BSA and Azide at 1.0mg/ml

Price (USD)

219.00

499.00

Printable

Key References

Bioinformatics

Formalin-fixed, paraffin-embedded human skeletal muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3677).

SDS-PAGE Analysis Purified Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3677). Confirmation of Purity and Integrity of Antibody.

Formalin-fixed, paraffin-embedded human skeletal muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3677).

SDS-PAGE Analysis Purified Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3677). Confirmation of Purity and Integrity of Antibody.

Formalin-fixed, paraffin-embedded human skeletal muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3677).

Product Details

Synonyms

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

Positive Control

Human skeletal muscle and heart muscle tissues (IHC).

Known Applications & Suggested Dilutions

ELISA, IHC-P More Details

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 °C followed by cooling at RT for 20 minutes)
Optimal dilution for a specific application should be determined.

Immunogen

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

Cellular Localization

Cell surface. Cytoplasm.

Species Reactivity

Human.

Host / Ig Isotype

Mouse IgG2b, kappa

Mol. Weight of Antigen

427kDa

Specificity & Comments

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.