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Product name
Anti-Dystrophin antibody
See all Dystrophin products (14) ...
Description
Rabbit polyclonal to Dystrophin
Specificity
The antibody also detects the short isoform Dp71
Tested applications
ICC/IF, IHC-Fr, WB, IHC-Pmore details
Cross reactivity
Reacts with
Mouse, Dog, Human
Predicted to work with
Rat
Immunogen
Synthetic peptide: EQLNNSFPSSRGRNTPG, corresponding to amino acids 3661-3677 of Human Dystrophin
EQLNNSFPSSRGRNTPG
Positive control
Skeletal muscle
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage buffer
pH: 7.40
Preservative: 0.1% Sodium azide
Constituents: 1% BSA, 0.0268% PBS
Concentration
Concentration information loading...
Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Research areas
Stem Cells >> Mesenchymal Stem Cells >> Myogenesis
Neuroscience >> Neurology process >> Neurodegenerative disease >> Other
Signal Transduction >> Cytoskeleton / ECM >> Cytoskeleton >> Microfilaments >> Actin etc >> Actin Binding Proteins
Applications
Show applications key
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.
ICC/IF: Use at an assay dependent dilution.
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.
Target
Function
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.
Tissue specificity
Expressed 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.
Involvement in disease
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 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 similarities
Contains 2 CH (calponin-homology) domains.
Contains 22 spectrin repeats.
Contains 1 WW domain.
Contains 1 ZZ-type zinc finger.
Cellular localization
Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
Target information above from: UniProt accessionP11532
The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).
Alternative names
- Apo dystrophin antibody
- BMD antibody
- CMD3B antibody
see all
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
Anti-Dystrophin antibody images:
Immunohistochemistry (Formalin-fixed paraffin-embedded sections) - Dystrophin antibody (ab15277)
Immunohistochemical staining of human skeletal muscle with ab15277
Immunohistochemistry (Frozen sections) - 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.
Immunocytochemistry/ Immunofluorescence - Dystrophin antibody (ab15277)
ab15277 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.
This image is courtesy of an anonymous Abreview
References for Anti-Dystrophin antibody (ab15277)
This product has been referenced in:
- Lawlor MW et al. Inhibition of activin receptor type IIB increases strength and lifespan in myotubularin-deficient mice. Am J Pathol 178:784-93 (2011). IHC-Fr; Mouse.Read more (PubMed: 21281811) »
- Kumar A et al. Muscle-specific expression of insulin-like growth factor 1 improves outcome in Lama2Dy-w mice, a model for congenital muscular dystrophy type 1A. Hum Mol Genet 20:2333-43 (2011). IHC; Mouse.Read more (PubMed: 21441569) »
See all 27 publications for this product
Publishing research using ab15277? Please let us know so that we can cite the reference in this datasheet
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"