Product nameAnti-Dystrophin antibody, prediluted
See all Dystrophin primary antibodies
DescriptionRabbit polyclonal to Dystrophin, prediluted
Tested applicationsSuitable for: IHC-Fr, IHC-Pmore details
Species reactivityReacts with: Human
Predicted to work with: Rat
- Skeletal muscle
Storage instructionsShipped at 4°C. Store at +4°C.
Storage bufferpH: 7.6
Preservative: 0.1% Sodium azide
Constituents: 99% Tris buffered saline, 1% BSA
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab15278 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
IHC-Fr: Use at an assay dependent dilution (PMID 18384691).
Not tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
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
Immunohistochemical staining of human skeletal muscle with ab15278
ab15278 staining Dystrophin in transverse sections of tibialis anterior (TA) muscles of mdx mice by Immunohistochemistry (Frozen sections). Mice were killed and TA muscles were removed, pinned to parafilm-covered cork, snap frozen in liquid N2-cooled isopentane, and stored at -80°C. Sections were cut using cryostat and blocked with 10% normal goat serum in 1% BSA/PBS for 1 h and then incubated with primary antibody for 1 hour. A Cy3 conjugated anti rabbit IgG at 1/500 dilution was used as secondary. Slides were coverslipped with Vectashield mounting medium with DAPI and imaged.
This product has been referenced in:
- Praissman JL et al. The functional O-mannose glycan on a-dystroglycan contains a phospho-ribitol primed for matriglycan addition. Elife 5:N/A (2016). IF . Read more (PubMed: 27130732) »
- Williams JH et al. Nanopolymers improve delivery of exon skipping oligonucleotides and concomitant dystrophin expression in skeletal muscle of mdx mice. BMC Biotechnol 8:35 (2008). IHC-Fr ; Mouse . Read more (PubMed: 18384691) »