- Datasheet
- Specific References (139)
- Protocols
Overview
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Product nameAnti-Dystrophin antibody
See all Dystrophin primary antibodies -
DescriptionRabbit polyclonal to Dystrophin
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Host speciesRabbit
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Tested applicationsSuitable for: ICC/IF, IHC-Fr, WB, IHC-P, IHC - Wholemountmore details
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Species reactivityReacts with: Mouse, Rat, Dog, Human
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Immunogen
Synthetic peptide within Human Dystrophin (C terminal). The exact sequence is proprietary.
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Positive control
- Skeletal muscle
Properties
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FormLiquid
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
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Storage bufferpH: 7.40
Preservative: 0.1% Sodium azide
Constituents: 1% BSA, 0.0268% PBS -
Concentration information loading... -
PurityImmunogen affinity purified
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ClonalityPolyclonal
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IsotypeIgG
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Research areas
Associated products
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Compatible Secondaries
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Immunohistochemistry kits
Applications
Our Abpromise guarantee covers the use of ab15277 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| ICC/IF | Use at an assay dependent concentration. | |
| IHC-Fr | 1/400. Use with acetone-fixed tissues. | |
| WB | Use a concentration of 1 µg/ml. Predicted molecular weight: 427 kDa. | |
| IHC-P | 1/100. | |
| IHC - Wholemount | Use at an assay dependent concentration. PubMed: 24852842 |
Target
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FunctionAnchors 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.
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Tissue specificityExpressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Isoform 5 is expressed in heart, brain, liver, testis and hepatoma cells. Most tissues contain transcripts of multiple isoforms, however only isoform 5 is detected in heart and liver.
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Involvement in diseaseDefects 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 cardiomyopathy dilated X-linked type 3B (CMD3B) [MIM:302045]; also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. -
Sequence similaritiesContains 2 CH (calponin-homology) domains.
Contains 22 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger. -
Cellular localizationCell membrane > sarcolemma. Cytoplasm > cytoskeleton.
- Information by UniProt
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Database links
- Entrez Gene: 1756 Human
- Entrez Gene: 13405 Mouse
- Entrez Gene: 24907 Rat
- Omim: 300377 Human
- SwissProt: O97592 Dog
- SwissProt: P11532 Human
- SwissProt: P11531 Mouse
- SwissProt: P11530 Rat
see all -
Alternative names
- BMD antibody
- CMD3B antibody
- DMD antibody
see all
Images
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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Dystrophin antibody (ab15277)Immunohistochemical staining of human skeletal muscle with ab15277 -
Immunohistochemistry (Frozen sections) - Anti-Dystrophin antibody (ab15277)Muscle stem cells (from normal mouse) were injected into the gastric muscle of an MDX mouse. Dystrophin staining: primary antibody ab15277 and secondary antibody is donkey anti-rabbit Alexa 594.
This image was kindly supplied as part of the review submitted by Jessica Tebbets.
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Immunocytochemistry/ Immunofluorescence - Anti-Dystrophin antibody (ab15277)This image is courtesy of an anonymous Abreviewab15277 staining dog muscle cells by ICC/IF. Cells were methanol fixed and incubated with ab15277, diluted 1/200, for 12 hours at 4°C. A FITC conjugated goat anti-rabbit antibody, diluted 1/200, was used as the secondary.
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Immunocytochemistry/ Immunofluorescence - Anti-Dystrophin antibody (ab15277)This image is courtesy of an anonymous AbreviewImmunofluorescence analysis of Human muscle fibers, staining Dystrophin with ab15277.
Cells were fixed with formaldehyde and blocked with 10% serum for 20 minutes at 23°C. Samples were incubated with primary antibody (1/100 in diluent) for 12 hours at 2°C. An AlexaFluor®488-conjugated goat anti-rabbit polyclonal IgG (1/200) was used as the secondary antibody.
Protocols
References
This product has been referenced in:
- Frustaci A et al. Novel a-Actin Gene Mutation p.(Ala21Val) Causing Familial Hypertrophic Cardiomyopathy, Myocardial Noncompaction, and Transmural Crypts. Clinical-Pathologic Correlation. J Am Heart Assoc 7:N/A (2018). Read more (PubMed: 29440008) »
- Tosic M et al. Lsd1 regulates skeletal muscle regeneration and directs the fate of satellite cells. Nat Commun 9:366 (2018). Read more (PubMed: 29371665) »
Customer reviews and Q&As
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