Key features and details
- Rabbit polyclonal to Dystrophin
- Suitable for: IHC-P
- Reacts with: Human
- Isotype: IgG
Product nameAnti-Dystrophin antibody
See all Dystrophin primary antibodies
DescriptionRabbit polyclonal to Dystrophin
Tested applicationsSuitable for: IHC-Pmore details
Species reactivityReacts with: Human
- IHC-P: Human heart muscle, skeletal muscle and prostate tissue.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: PBS, 40% Glycerol (glycerin, glycerine)
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab251665 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
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.
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.
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
- BMD antibody
- CMD3B antibody
- DMD antibody
Formalin-fixed, paraffin-embedded human heart muscle tissue stained for Dystrophin with ab251665 at a 1/200 dilution in immunohistochemical analysis.
Formalin-fixed, paraffin-embedded human skeletal muscle tissue stained for Dystrophin with ab251665 at a 1/200 dilution in immunohistochemical analysis.
Formalin-fixed, paraffin-embedded human heart muscle tissue shows an absence in immunoreactivity for Dystrophin when incubated with ab251665 at a 1/200 dilution (negative control).
Formalin-fixed, paraffin-embedded human prostate tissue stained for Dystrophin with ab251665 at a 1/200 dilution in immunohistochemical analysis.
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab251665 has not yet been referenced specifically in any publications.