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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| Ch, H, M, R | WB, IHC | M | Purified | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB1645 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-Dystrophin Antibody, clone 1808 |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Liquid in 0.02 M phosphate buffer (pH 7.6), 250 mM NaCl with 0.1% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Dystrophin Antibody, clone 1808 detects level of Dystrophin & has been published & validated for use in WB, IH. |
| Key Applications |
|
| Application Notes | Western blot Immunohistochemistry Optimal working dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Peripheral membrane proteins extracted from Torpedo postsynaptic membranes. |
| Clone | 1808 |
| Host | Mouse |
| Specificity | By Western blot MAB1645 recognizes a single protein having a relative molecular weight of 300 kD. Reacts to mammalian skeletal muscle dystrophin. Shows no staining of mdx mouse muscle. Labels normal human muscle but not Duchenne muscle. STAINING PATTERN: On cryostat sections of normal mouse diaphragm MAB1645 gives strong immunofluorescence staining of the entire sarcolemma, with particularly strong staining of neuromuscular junctions. SPECIES REACTIVITY: Labels skeletal muscle from rat, chicken and Xenopus laevis. Labels cardiac muscle from rat and normal, but not mdx, mouse. Also labels smooth muscle from chicken gizzard. |
| Isotype | IgG1 |
| 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 |
|
| UniProt Number |
|
| 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 | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20ºC for up to 12 months in convenient aliquots. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |