製品の概要

  • 製品名Anti-Dysferlin antibody
    Dysferlin 一次抗体 製品一覧
  • 製品の詳細
    Rabbit polyclonal to Dysferlin
  • アプリケーション適用あり: IHC-P, WBmore details
  • 種交差性
    交差種: Human
    交差が予測される動物種: Orangutan
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 2050 to the C-terminus of Human Dysferlin.

    (Peptide available as ab97417.)

  • ポジティブ・コントロール
    • This antibody gave a positive signal in Human skeletal muscle tissue lysate.

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • バッファーPreservative: 0.02% Sodium Azide
    Constituents: 1% BSA, PBS, pH 7.4
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • ポリ/モノポリクローナル
  • アイソタイプIgG
  • 研究分野

アプリケーション

Our Abpromise guarantee covers the use of ab85802 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

アプリケーション Abreviews 特記事項
IHC-P Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 248 kDa (predicted molecular weight: 237 kDa).

ターゲット情報

  • 機能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.
  • 組織特異性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.
  • 関連疾患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.
  • 配列類似性Belongs to the ferlin family.
    Contains 5 C2 domains.
  • 発生段階Expression in limb tissue from 5-6 weeks embryos; persists throughout development.
  • ドメインThe C2 domain 1 associates with lipid membranes in a calcium-dependent manner.
  • 細胞内局在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
  • 参照データベース
  • 別名
    • 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 画像

  • Anti-Dysferlin antibody (ab85802) 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
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 237 kDa
    Observed band size : 248 kDa (why is the actual band size different from the predicted?)
    Additional bands at : 31 kDa,44 kDa,61 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 5 minutes
  • IHC image of Dysferlin staining in human skeletal muscle formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab85802, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

Anti-Dysferlin antibody (ab85802) 使用論文

ab85802 has not yet been referenced specifically in any publications.

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