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Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody [DMD/6270]

In Stock
Catalog Number Formulation Size Price
1756-MSM8-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
1756-MSM8-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
1756-MSM8-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $60 | Orders outside the US - Contact Us for Order Information | Ships next business day

Applications & Dilutions

Applications Tested Dillution Protocol Note
Immunohistochemistry (IHC)
1-2ug/ml
30 min 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

Summary

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 Properties & Targets

Antibody Type
Host
Mouse
Applications
Species Reactivity
Research Areas
Isotype / Light Chain
IgG2a / Kappa
Cellular Localization
Cell junction, Cell membrane, Cytoplasm, Cytoskeleton, Postsynaptic cell membrane, Sarcolemma, Synapse
Gene Name
DMD
Positive Control
Human skeletal muscle and heart muscle tissues (IHC).
Immunogen
Recombinant fragment (around aa1700-2300) of human DMD (exact sequence is proprietary).
Alternate Names
Dystrophin, BMD; CMD3B; Duchenne muscular dystrophy (DMD); Dystrophin; Muscular dystrophy Duchenne and Becker types

Database Links

Entrez Gene ID
SwissProt

Additional Information

Clone
DMD/6270
Chromosome Location
Xp21.2
Mol. Weight of Antigen
427kDa

Functions

  • Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin-associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission.

Key References

  • BlakeDJ, et al. (2002)Function and genetics of dystrophin and dystrophin related proteins in muscle.Physiol Rev82:291 329.

PubMed Links

Storage & Stability

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. No MSDS required.

Limitations

This antibody is available for research use only and is not approved for use in diagnosis.

Supplied as

200ug/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.

Warranty

There are no warranties, expressed or implied, which extend beyond this description. Company is not liable for any personal injury or economic loss resulting from this product.

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