Overview

  • Product name
  • Description
    Rabbit polyclonal to Dystrophin
  • Host species
    Rabbit
  • Tested applications
    Suitable for: ICC/IF, IHC-Fr, WB, IHC-P, IHC - Wholemountmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Dog, Human
    Predicted to work with: Pig
  • Immunogen

    Synthetic peptide within Human Dystrophin aa 3650 to the C-terminus (C terminal). The exact sequence is proprietary.
    Database link: P11532

  • Positive control
    • Skeletal muscle

Properties

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

  • 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.
  • Information by UniProt
  • Database links
  • Alternative names
    • BMD antibody
    • CMD3B antibody
    • DMD antibody
    • DMD_HUMAN antibody
    • Duchenne muscular dystrophy protein antibody
    • Dystrophin antibody
    • Muscular dystrophy Duchenne and Becker types antibody
    see all

Images

  • Immunohistochemical staining of human skeletal muscle with 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.

  • 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.

    See Abreview

  • Immunofluorescence 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.

    See Abreview

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) »

See all 140 Publications for this product

Customer reviews and Q&As

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunocytochemistry/ Immunofluorescence
Sample
Human Cell (Muscle Fiber)
Specification
Muscle Fiber
Fixative
Formaldehyde
Permeabilization
No
Blocking step
Serum as blocking agent for 20 minute(s) · Concentration: 10% · Temperature: 23°C
Username

Abcam user community

Verified customer

Submitted Dec 19 2012

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Pig Tissue sections (Cardiac muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Cardiac muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Rat Tissue sections (Cardiac muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Cardiac muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Sheep Tissue sections (Cardiac muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Cardiac muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Mouse Tissue sections (Skeletal muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Skeletal muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Common marmoset Tissue sections (Skeletal muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Skeletal muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Dog Tissue sections (Skeletal muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Skeletal muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Cat Tissue sections (Myocardium)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Myocardium
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Human Tissue sections (Heart muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: Citric acid
Permeabilization
No
Specification
Heart muscle
Blocking step
BSA as blocking agent for 10 minute(s) · Concentration: 2% · Temperature: 21°C
Fixative
Formaldehyde
Username

Mr. Carl Hobbs

Verified customer

Submitted Jul 27 2018

Abcam guarantees this product to work in the species/application used in this Abreview.
Application
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample
Mouse Tissue sections (Muscle)
Antigen retrieval step
Heat mediated - Buffer/Enzyme Used: pH 6.0 Citrate
Permeabilization
No
Specification
Muscle
Blocking step
PB ab64226 as blocking agent for 10 minute(s) · Concentration: 1% · Temperature: RT°C
Fixative
Formaldehyde
Username

Abcam user community

Verified customer

Submitted Nov 22 2017

1-10 of 34 Abreviews or Q&A

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