概述

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

    Synthetic peptide (unfortunately, the amino acid sequence is considered to be commercially sensitive) (Human) (C terminal).

  • 阳性对照
    • Skeletal muscle.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • 存储溶液Preservative: 0.1% Sodium Azide
    Constituents: 1% BSA, 10mM PBS, pH 7.4
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab15108 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
WB Use a concentration of 1 µg/ml. Predicted molecular weight: 231 kDa.
IHC-P 1/50.
ICC/IF Use at an assay dependent concentration.

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 图像

  • ab15108 staining Dysferlin in human skeletal muscle by Immunohistochemistry (FFPE-sections).

  • Anti-Dysferlin antibody (ab15108) at 1 µg/ml + Human skeletal muscle tissue lysate - total protein (ab29330) at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution

    Predicted band size : 231 kDa
    Observed band size : 231 kDa
    Additional bands at : 240 kDa (possible post-translational modification).

Anti-Dysferlin antibody (ab15108)参考文献

This product has been referenced in:
  • Guo LT  et al. Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle. Neuromuscul Disord : (2010). WB ; Human, Mouse, Dog . Read more (PubMed: 20817457) »
  • Peter AK  et al. Myogenic Akt signaling upregulates the utrophin-glycoprotein complex and promotes sarcolemma stability in muscular dystrophy. Hum Mol Genet 18:318-27 (2009). Read more (PubMed: 18986978) »

See all 3 Publications for this product

Product Wall

Application Western blot
Loading amount 50 µg
Gel Running Conditions Reduced Denaturing (4-12% Bis-Tris gel)
Sample Sheep Tissue lysate - whole (Right atrial appendage)
Specification Right atrial appendage
Blocking step SuperBlock as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 100% · Temperature: 22°C
Username

Abcam user community

Verified customer

提交于 Apr 03 2014

Thank you for submitting an Abreview for this product.

I am sorry to see that this product did not perform as expected in WB on canine and human samples. We guarantee our products to work as stated on the datasheet and would appreciate the o...

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Abreviews
Application Western blot
Sample Human Cell lysate - whole cell (HeLa)
Loading amount 25 µg
Specification HeLa
Gel Running Conditions Reduced Denaturing (Bio-Rad/TGX/Any kD)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Abcam user community

Verified customer

提交于 Dec 04 2012

Abcam guarantees this product to work in the species/application used in this Abreview.
Application Immunocytochemistry/ Immunofluorescence
Sample Dog Cell (Muscle)
Specification Muscle
Fixative Acetone
Username

Abcam user community

Verified customer

提交于 Jul 21 2008

Thank you for your enquiry. Since you are using 4% PFA, which also contains formalin, I would suggest doing the antigen retrieval step. Formaldehyde fixes not by coagulation, but by reacting primarily with basic amino acids to form cross-linking "m...

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Thank you for your enquiry. The antigen sequence is exactly same as that of mouse (100%). I hope this information helps. Please do not hesitate to contact us again if you need anything further.

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