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

Additional information

Catalog Number: 1756-RBM10
Gene:

Host:

Reactivity:

In Stock

Trial Size Available

Carrier-Free Available

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In Stock
Catalog Number Formulation Size Price
1756-RBM10-P0
Purified Ab with BSA and Azide at 200ug/ml
20ug
$259.00
1756-RBM10-P1
Purified Ab with BSA and Azide at 200ug/ml
100ug
$559.00
1756-RBM10-P1ABX
Purified Ab WITHOUT BSA or Azide at 1.0mg/ml
100ug
$559.00
Flat Rate Domestic: $95 | 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.

  • IHC
  • WB
  • Documents
  • Applications & Dilutions
  • Product Properties
  • other information

Documents

Document Name
Datasheet Download Here
Material Safety Data Sheet Download Here

Applications & Dilutions

Applications Tested Dilutions Protocol Note
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

Product Properties

Antibody Type
Recombinant Rabbit Monoclonals
Host
Rabbit
Applications
Immunohistochemistry, Western Blot
Species Reactivity
Human
Clone
DMD/8773R
Isotype
IgG
Light Chain
Kappa
Cellular Localization
Cell membrane, Cytoplasm, Cytoskeleton, Postsynaptic cell membrane, Sarcolemma
Gene Name
DMD
Positive Control
Human skeletal muscle and heart muscle tissues (IHC).
Immunogen
Recombinant fragment (around aa2063-2078) of the human Dystrophin protein (exact sequence is proprietary)
Expression System
CHO
Purification
Protein A
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.
Supplied as
200ug/ml of Ab produced in a mammalian-based expression system. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.
Limitations
This antibody is available for research use only and is not approved for use in diagnosis.

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Other Information

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.
PubMed Links
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.
Entrez Gene ID
1756
SwissProt
P11532
Chromosome Location
Xp21.2-p21.1
Mol. Weight of Antigen
426.77kDa
Alternate Names
Dystrophin, BMD|CMD3B|DXS142|DXS164|DXS206|DXS230|DXS239|DXS268|DXS269|DXS270|DXS272|MRX85

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.

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