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Recombinant Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody [DMD/8773R]

In Stock
Catalog Number Formulation Size Price
1756-RBM10-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$229.00
1756-RBM10-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$519.00
1756-RBM10-P1ABX
Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
100ug
$519.00
Flat Rate Domestic: $75 | Orders outside the US - Contact Us for Order Information | Ships next business day
Recombinant Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody

Formalin-fixed, paraffin-embedded human skeletal muscle stained with Dystrophin Recombinant Rabbit Monoclonal Antibody (DMD/8773R). HIER: Tris/EDTA, pH9.0, 45min. 2°C: HRP-polymer, 30min. DAB, 5min.

Recombinant Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody - Image 2

SDS-PAGE Analysis of Purified Dystrophin Recombinant Rabbit Monoclonal Antibody (DMD/8773R) Confirmation of Purity and Integrity of Antibody.

Applications & Dilutions

Applications Tested Dillution Protocol Note
Immunofluorescence (IF)
1-3ug/ml
Immunohistochemistry (IHC)
1-2ug/ml
IHC Protocol
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
Western Blot (WB)
2-4ug/ml
Western Blot Protocol

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
Recombinant Rabbit Monoclonals
Host
Rabbit
Applications
Immunofluorescence, Immunohistochemistry, Western Blot
Species Reactivity
Human
Research Areas
Cardiovascular
Isotype / Light Chain
IgG / Kappa
Cellular Localization
Cell Surface. Cytoplasm.
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
1756
SwissProt
P11532

Additional Information

Clone
DMD/8773R
Chromosome Location
Xp21.2
Mol. Weight of Antigen
427kDa

Related Antibodies

View All DMD Antibodies

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 dystrophinrelated 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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