概述

  • 产品名称Anti-Dystrophin抗体
    参阅全部 Dystrophin 一抗
  • 描述
    兔多克隆抗体to Dystrophin
  • 经测试应用适用于: ICC/IF, IHC-Fr, WB, IHC-P, IHC - Wholemountmore details
  • 种属反应性
    与反应: Mouse, Rat, Dog, Human
  • 免疫原

    Synthetic peptide:

    EQLNNSFPSSRGRNTPG

    , corresponding to amino acids 3661-3677 of Human Dystrophin

  • 阳性对照
    • Skeletal muscle

性能

应用

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.

应用 Ab评论 说明
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

靶标

  • 功能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.
  • 组织特异性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.
  • 疾病相关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.
  • 序列相似性Contains 2 CH (calponin-homology) domains.
    Contains 22 spectrin repeats.
    Contains 1 WW domain.
    Contains 1 ZZ-type zinc finger.
  • 细胞定位Cell membrane > sarcolemma. Cytoplasm > cytoskeleton.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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

Anti-Dystrophin antibody 图像

  • 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

Anti-Dystrophin antibody (ab15277)参考文献

This product has been referenced in:
  • Vanhoutte D  et al. Thrombospondin expression in myofibers stabilizes muscle membranes. Elife 5:N/A (2016). IHC-P ; Mouse . Read more (PubMed: 27669143) »
  • Baradaran-Heravi A  et al. Novel small molecules potentiate premature termination codon readthrough by aminoglycosides. Nucleic Acids Res 44:6583-98 (2016). Read more (PubMed: 27407112) »

See all 61 Publications for this product

Product Wall

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

提交于 Dec 19 2012

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Human Tissue sections (Skeletal muscle)
Antigen retrieval step Enzymatic - Buffer/Enzyme Used: 0.05% trypsin
Permeabilization No
Specification Skeletal muscle
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 23°C
Fixative Formaldehyde
Username

Abcam user community

Verified customer

提交于 Feb 09 2016



I apologize for the delay in my reply. We do not have any experimental data regarding the cross reactivity of ab15277 with human Utrophin. The immunogen for ab15277 shares no homology with the human Utrophin sequence.

Application Immunohistochemistry (Frozen sections)
Blocking step Dako Protein Blocking Buffer as blocking agent for 30 minute(s) · Concentration: 0.25% · Temperature: 21°C
Sample Rat Tissue sections (Aorta)
Specification Aorta
Permeabilization No
Fixative Acetone
Username

Dr. Charlene Blake

Verified customer

提交于 Feb 12 2014

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 (Skeletal muscle)
Specification Skeletal muscle
Fixative Paraformaldehyde
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: pH 6, citrate based 20 min in microwave
Permeabilization Yes - 0.1% Tween 20
Blocking step 2%BSA + 5%NGS in PBS + 0.1% Tween20 as blocking agent for 45 minute(s) · Concentration: 100% · Temperature: 25°C
Username

Mr. Josh Meisner

Verified customer

提交于 Apr 30 2013



I have been discussing this with the lab, and they actually use a 1:25 dilution of this antibody in Western blot. Would you be able to try this high concentration of antibody, to see if the specific signal improves? I have attached the Wester...

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Thank you for your phone enquiries and interest in our products.

ab7163 - Anti-Dystrophin antibody [MANDYS8]

This antibody Monoclonal Anti-Dystrophin, clone MANDYS8, recognizes an epitope located on the rod domain of the human dyst...

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Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunohistochemistry (Frozen sections)
Sample Mouse Tissue sections (Muscle)
Specification Muscle
Fixative Formaldehyde
Permeabilization No
Blocking step Serum as blocking agent for 30 minute(s) · Concentration: 10% · Temperature: RT°C
Username

Abcam user community

Verified customer

提交于 May 02 2012

Thank you for contacting us. Because we carry over 70,000 products, it isn't feasible for us to keep small sample sizes of our products.

We are happy to reassure our customers that all of our products are covered by our Abpromise, which guar...

Read More
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 (gastrocnemius)
Specification gastrocnemius
Fixative Formaldehyde
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Proteinase K
Permeabilization No
Blocking step BSA as blocking agent for 25 minute(s) · Concentration: 1% · Temperature: 25°C
Username

Abcam user community

Verified customer

提交于 Oct 03 2011

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