RabMAb

Anti-Dysferlin抗体[JAI-1-49-3] (ab124684)

概述

  • 产品名称Anti-Dysferlin抗体[JAI-1-49-3]
    参阅全部 Dysferlin 一抗
  • 描述
    兔单克隆抗体[JAI-1-49-3] to Dysferlin
  • 经测试应用适用于: ICC/IF, IHC-Fr, WB, IHC-Pmore details
    不适用于: Flow Cyt
  • 种属反应性
    与反应: Mouse, Human
  • 免疫原

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) corresponding to Human Dysferlin aa 100-200.
    Database link: O75923

  • 阳性对照
    • WB: Human and mouse skeletal muscle tissue lysates. IHC-P: Human skeletal muscle tissue. IHC-Fr: Human and mouse skeletal muscle tissues. ICC/IF: A673 cells.
  • 常规说明

     

    This antibody was made in collaboration with the Jain Foundation whose goal is to hasten EVERY avenue that may lead to the cure for LGMD2B/Miyoshi.

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated ‘PUR’ on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.

     

    Produced using Abcam's RabMAb® technology. RabMAb® technology is covered by the following U.S. Patents, No. 5, 675, 063 and/or 7, 429, 487.

    This product is a recombinant rabbit monoclonal antibody.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Avoid freeze / thaw cycle.
  • 解离常数(KD KD = 7.20 x 10 -11 M Learn more about KD
  • 存储溶液pH: 7.20
    Preservative: 0.01% Sodium azide
    Constituents: 40% Glycerol, 0.05% BSA, 59% PBS
  • Concentration information loading...
  • 纯度Protein A purified
  • 克隆单克隆
  • 克隆编号JAI-1-49-3
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab124684 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 1/200 - 1/400.

 

IHC-Fr 1/200.
WB 1/1000. Detects a band of approximately 280 kDa (predicted molecular weight: 237 kDa).

For unpurified use at 1/1000 - 1/10000.

IHC-P 1/500. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

See protocols (link: http://www.abcam.com/protocols/ihc-antigen-retrieval-protocol).

For unpurified use at 1/50 - 1/100.

  • 应用说明Is unsuitable for Flow Cyt.
  • 靶标

    • 功能Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress.
    • 组织特异性Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas.
    • 疾病相关Defects in DYSF are the cause of limb-girdle muscular dystrophy type 2B (LGMD2B) [MIM:253601]. LGMD2B is an autosomal recessive degenerative myopathy characterized by weakness and atrophy starting in the proximal pelvifemoral muscles, with onset in the late teens or later, massive elevation of serum creatine kinase levels and slow progression. Scapular muscle involvement is minor and not present at onset. Upper limb girdle involvement follows some years after the onset in lower limbs.
      Defects in DYSF are the cause of Miyoshi muscular dystrophy type (MMD1) [MIM:254130]. MMD1 is a late-onset muscular dystrophy involving the distal lower limb musculature. It is characterized by weakness that initially affects the gastrocnemius muscle during early adulthood. Otherwise the phenotype overlaps with LGMD2B, especially in age at onset and creatine kinase elevation.
      Defects in DYSF are the cause of distal myopathy with anterior tibial onset (DMAT) [MIM:606768]. Onset of the disorder is between 14 and 28 years of age and the anterior tibial muscles are the first muscle group to be involved. Inheritance is autosomal recessive.
    • 序列相似性Belongs to the ferlin family.
      Contains 5 C2 domains.
    • 发展阶段Expression in limb tissue from 5-6 weeks embryos; persists throughout development.
    • 结构域The C2 domain 1 associates with lipid membranes in a calcium-dependent manner.
    • 细胞定位Cell membrane > sarcolemma. Cytoplasmic vesicle membrane. Colocalizes, during muscle differentiation, with BIN1 in the T-tubule system of myotubules and at the site of contact between two myotubes or a myoblast and a myotube. Wounding of myotubes led to its focal enrichment to the site of injury and to its relocalization in a Ca(2+)-dependent manner toward the plasma membrane. Colocalizes with AHNAK, AHNAK2 and PARVB at the sarcolemma of skeletal muscle. Detected on the apical plasma membrane of the syncytiotrophoblast. Reaches the plasmma membrane through a caveolin-independent mechanism. Retained by caveolin at the plasmma membrane (By similarity). Colocalizes, during muscle differentiation, with CACNA1S in the T-tubule system of myotubules (By similarity). Accumulates and colocalizes with fusion vesicles at the sarcolemma disruption sites.
    • Information by UniProt
    • 数据库链接
    • 别名
      • DMAT antibody
      • DYSF antibody
      • DYSF_HUMAN antibody
      • Dysferlin antibody
      • Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody
      • Dysferlin limb girdle muscular dystrophy 2B antibody
      • Dystrophy associated fer 1 like 1 antibody
      • Dystrophy associated fer 1 like protein antibody
      • Dystrophy associated fer1 like 1 antibody
      • Dystrophy associated fer1 like protein antibody
      • Dystrophy-associated fer-1-like protein antibody
      • Fer 1 like protein 1 antibody
      • Fer-1-like protein 1 antibody
      • Fer1 like protein 1 antibody
      • FER1L1 antibody
      • FLJ00175 antibody
      • FLJ90168 antibody
      • LGMD 2B antibody
      • LGMD2B antibody
      • Limb girdle muscular dystrophy 2B (autosomal recessive) antibody
      • Limb girdle muscular dystrophy 2B antibody
      • Miyoshi myopathy antibody
      • MM antibody
      • MMD1 antibody
      see all

    Anti-Dysferlin antibody [JAI-1-49-3] 图像

    • Immunohistochemistry (Frozen sections) analysis of unfixed frozen human skeletal muscle tissue (control) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with unpurified ab124684 at a dilution of 1/50.

    • Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (purified) + Mouse muscle tissue lysate at 10 µg

      Secondary
      Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution

      Predicted band size : 237 kDa
      Observed band size : 280 kDa (why is the actual band size different from the predicted?)

      Blocking and dilution buffer: 5% NFDM/TBST.

    • Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (purified) + Human skeletal muscle tissue lysate at 20 µg

      Secondary
      Peroxidase-conjugated goat anti-rabbit IgG, (H+L) at 1/1000 dilution

      Predicted band size : 237 kDa
      Observed band size : 280 kDa (why is the actual band size different from the predicted?)

      Blocking and dilution buffer: 5% NFDM/TBST.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human skeletal muscle tissue labelling Dysferlin with purified ab124684 at 1/500. Heat mediated antigen retrieval was performed using Tris/EDTA buffer pH 9. ab97051, a HRP-conjugated goat anti-rabbit IgG (H+L) was used as the secondary antibody (1/500). Negative control using PBS instead of primary antibody. Counterstained with hematoxylin.

    • Immunocytochemistry/Immunofluorescence analysis of A673 cells labelling Dysferlin with purified ab124684 at 1/300. Cells were fixed with 4% paraformaldehyde and permeabilized with 0.1% Triton X-100. ab150077, an Alexa Fluor® 488-conjugated goat anti-rabbit IgG (1/500) was used as the secondary antibody. DAPI (blue) was used as the nuclear counterstain. ab7291, a mouse anti-tubulin (1/1000) and ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500) were also used.

      Control 1: primary antibody (1/300) and secondary antibody, ab150120, an Alexa Fluor® 594-conjugated goat anti-mouse IgG (1/500).

      Control 2: ab7291 (1/1000) and secondary antibody, ab150077, an Alexa Fluor® 488-conjugated goat anti-rabbit IgG (1/500).

    • All lanes : Anti-Dysferlin antibody [JAI-1-49-3] (ab124684) at 1/1000 dilution (unpurified)

      Lane 1 : Human skeletal muscle tissue lysate (control)
      Lane 2 : Human skeletal muscle tissue lysate (LGMD2B)
      Lane 3 : Mouse skeletal muscle tissue lysate (wild-type mice)
      Lane 4 : Mouse skeletal muscle tissue lysate (Dysf-/- transgenic mouse)

      Lysates/proteins at 10 µg per lane.

      Secondary
      HRP-conjugated goat anti-rabbit IgG at 1/2000 dilution

      Predicted band size : 237 kDa
      Observed band size : 280 kDa (why is the actual band size different from the predicted?)
    • Immunohistochemistry (Frozen sections) analysis of unfixed frozen human skeletal muscle tissue (LGMD2B) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.

    • Immunohistochemistry (Frozen sections) analysis of unfixed frozen mouse skeletal muscle tissue (wild type) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.

    • Immunohistochemistry (Frozen sections) analysis of unfixed frozen mouse skeletal muscle tissue (Dysf-/- transgenic mouse) labelling Dysferlin with unpurified ab124684 at a dilution of 1/200.

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    Anti-Dysferlin antibody [JAI-1-49-3] (ab124684)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). WB ; Mouse . Read more (PubMed: 27669143) »
    • Tzeng HP  et al. Dysferlin mediates the cytoprotective effects of TRAF2 following myocardial ischemia reperfusion injury. J Am Heart Assoc 3:e000662 (2014). WB ; Mouse . Read more (PubMed: 24572254) »

    See all 8 Publications for this product

    Product Wall

    Application Western blot
    Loading amount 40 µg
    Gel Running Conditions Reduced Denaturing (3-8%)
    Sample Human Cell lysate - whole cell (Skeletal muscle cells)
    Specification Skeletal muscle cells
    Blocking step Milk as blocking agent for 5 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
    Username

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    提交于 Oct 27 2014

    Application Western blot
    Loading amount 100 µg
    Gel Running Conditions Reduced Denaturing (7% Tris-Acetate Protein Gel in Tris-Acetate SDS Running Buffer)
    Sample Sheep Tissue lysate - whole (Left atrial appendage)
    Specification Left atrial appendage
    Blocking step SuperBlock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 23°C
    Username

    Abcam user community

    Verified customer

    提交于 Feb 07 2014

    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"