Thermo Fisher Scientific Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Monoclonal Antibody (DMD/3676)

Applications

Tested Dilution

Publications

Immunohistochemistry (Paraffin) (IHC (P))

1-2 µg/mL

-

ELISA (ELISA)

Assay-dependent

-

Peptide Array (Array)

Assay-dependent

-

Immunoelectrophoresis (IE)

Assay-dependent

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2b, kappa

Class

Monoclonal

Type

Antibody

Clone

DMD/3676

Immunogen

A recombinant fragment (around aa 114-263) of human DMD protein. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:Dystrophin,uniProtId:P11532-1,ncbiNodeId:9606,antigenRange:114-263,antigenLength:3685,antigenImageFileName:1756-MSM6-P1_Dystrophin_P11532-1_House_mouse.svg,antigenImageFileNamePDP:1756-MSM6-P1_Dystrophin_P11532-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

200 µg/mL

Purification

Protein A/G

Storage buffer

PBS with 0.05% BSA

Contains

0.05% sodium azide

Storage conditions

4° C

Shipping conditions

Ambient (domestic); Wet ice (international)

Product Specific Information

Antibody is stable for 24 months.

Positive Control: Human skeletal muscle and heart muscle tissues (IHC). Cellular Localization: Cell surface. Cytoplasm.

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.

Target Information

Dystrophin is the 427kDa protein product of the DMB/BMD gene located on the X chromosome at position Xp21. Western blotting and immunohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies. Dystrophin abnormalities are thought to occur in 100% of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65% of cases.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.