| Product Name | Dystrophin antibody |
|---|---|
| Antibody Type | Primary Antibodies |
| Product description | Mouse monoclonal [0.N.258] to Dystrophin - BSA and Azide freeThe dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X linked disorder occurring at a frequency of about 1 in 3,500 new born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue specific promoters and two polyA addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin glycoprotein complex (DGC), which bridges the inner cytoskeleton (F actin) and the extracellular matrix. |
| Immunogen | Acetylcholine receptor (AChR) enriched membranes from Torpedo nobiliana electric organ. |
| Clonality | Monoclonal |
|---|---|
| Isotype | IgG1 |
| Host Species | Mouse |
| Tested Applications | PurifyEMELISAIHC-FIHC-P |
| AP: Use at an assay dependent dilution. Electron Microscopy: Use at an assay dependent dilution. ELISA: Use at an assay dependent dilution. IHC-P: Use at a concentration of 2 - 4 μg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. Boil tissue sections in 1mM EDTA, pH 8.0, for 10-20 min followed by cooling at RT for 20 min. Note that 1mM EDTA, pH 8.0 is better than 10mM citrate buffer, pH 6.0 for unmasking the epitope. IHC-Fr: Use at a concentration of 2 - 4 μg/ml. Optimal dilutions/concentrations should be determined by the end user.: |
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| Species Reactivity | HumanMouseRatTorpedoXenopus |
| Concentration | 0.5mg/ml |
| Purification | Protein G |
| Gene Synonyms | Dystrophin DXS269 DXS272 1756 DXS268 DXS239 DXS270 DXS142 300376 DXS206 P11532 DXS230 DMD DXS164 BMD CMD3B |
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| Application Notes | AP: Use at an assay dependent dilution. Electron Microscopy: Use at an assay dependent dilution. ELISA: Use at an assay dependent dilution. IHC-P: Use at a concentration of 2 - 4 μg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol. Boil tissue sections in 1mM EDTA, pH 8.0, for 10-20 min followed by cooling at RT for 20 min. Note that 1mM EDTA, pH 8.0 is better than 10mM citrate buffer, pH 6.0 for unmasking the epitope. IHC-Fr: Use at a concentration of 2 - 4 μg/ml. Optimal dilutions/concentrations should be determined by the end user.: |
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| Form | Liquid |
|---|---|
| Storage Instructions | Keep as concentrated solution. Store at 4ºC short term. For extended storage aliquot and store at -20ºC or below. Avoid freeze-thaw cycles. |
| Storage Buffer | Preservative: NoneConstituents: 10mM PBS. pH 7.4 |
