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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| Ca, H, M, R, Rb | WB, IHC | M | Culture Supernatant | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB1692 |
| Brand Family | Chemicon® |
| Trade Name |
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| Description | Anti-Dystrophin Antibody, mid-rod, clone 6D3 |
| Product Information | |
|---|---|
| Format | Culture Supernatant |
| Control |
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| Presentation | Culture supernatant, liquid in PBS with 1% BSA, containing 15 mM sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-Dystrophin Antibody, mid-rod, clone 6D3 is validated for use in WB, IH for the detection of Dystrophin. |
| Key Applications |
|
| Application Notes | Immunohistochemistry: (fresh frozen, unfixed tissue only): use undiluted - 1:20. Not recommended for use on paraffin embedded tissue. EM Gold (Light fixation with 2% formaldehyde + 0.001% glutaraldehyde for 1 hour. 2.3M sucrose used as cryoprotectant.): use undiluted. 90 minute incubation at 25°C. Western blotting: use 1:100-1:250. Optimal working dilutions must be determined by the end user. Protocol for Immunohistochemical use of MAB1692 1) Freeze muscle blocks in isopentane chilled in liquid nitrogen. 2) Cut 4 μm to 10 μm sections and air dry on slides coated with 0.5% gelatin containing 0.05% chrome alum. 3) Slides may be stored at -70 °C wrapped in cling film until required. If stored sections are used, allow sections to equilibrate to room temperature before unwrapping and proceeding. 4) Apply a 50 μL aliquot of primary antibody to sections (unfixed). Incubate for 1 hour at room temperature or 37°C. 5) Wash sections 3 x 10 minutes in phosphate buffered saline. 6) Apply a 50 μL aliquot of labeled second antibody. Incubate for 60 minutes at 25°C. 7) Wash sections 3 x 10 minutes in phosphate buffered saline. 8) Mount fluorescent sections in aqueous mounting media or visualize peroxidase label (DAB). Dehydrate, clean and mount peroxidase labeled sections for permanent preparations. |
| Biological Information | |
|---|---|
| Immunogen | Bacterial fusion protein (Cell (1987) 51:919-928). |
| Epitope | mid-rod |
| Clone | 6D3 |
| Host | Mouse |
| Specificity | Mid rod domain (between amino acids 1181 and 1388) of human dystrophin. Also reacts with skeletal, cardiac and smooth muscle dystrophin from normal mouse, rat, rabbit and dog. Other animal species have not been tested. Reacts on blots with the brain isoform. No reactivity with mdx mouse tissue or DMD/BMD patients who have a gene deletion which removes the antibody binding site. Does not react with chicken dystrophin. STAINING PATTERN:Light microscopy: continuous rim of labeling at the periphery of muscle fibers. E.M. gold: close to the cytoplasmic face of the plasma membrane. Western blotting: strong double bands at approximately 400 kD plus metabolites of lower molecular mass. |
| Isotype | IgG2a |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | The 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 extra-cellular matrix. |
| Gene Symbol |
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| UniProt Number |
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| UniProt Summary | FUNCTION: SwissProt: P11532 # May play a role in anchoring the cytoskeleton to the plasma membrane. SIZE: 3685 amino acids; 426692 Da SUBUNIT: Interacts with the syntrophins SNTA1, SNTB1, SNTB2, SNTG1 and SNTG2. Interacts with KRT19. SUBCELLULAR LOCATION: Cell membrane, sarcolemma; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton. TISSUE SPECIFICITY: Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Isoform 5 is expressed in brain, liver, testis and hepatoma cells. DISEASE: SwissProt: P11532 # Defects in DMD are the cause of Duchenne muscular dystrophy (DMD) [MIM:310200]. DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. & Defects in DMD are the cause of Becker muscular dystrophy (BMD) [MIM:300376]. BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. & Defects in DMD are a cause of dilated cardiomyopathy [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dystrophin mutations may predispose to common sporadic cardiomyopathy cases. SIMILARITY: SwissProt: P11532 ## Contains 2 CH (calponin-homology) domains. & Contains 22 spectrin repeats. & Contains 1 WW domain. & Contains 1 ZZ-type zinc finger. MISCELLANEOUS: The DMD gene is the largest known gene in humans. It is 2.4 million base-pairs in size, comprises 79 exons and takes over 16 hours to be transcribed and cotranscriptionally spliced. |
| Product Usage Statements | |
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| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C for up to one year in convenient undiluted aliquots. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 1 mL |