製品の概要

  • 製品名Anti-FHL1 antibody
    FHL1 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to FHL1
  • 特異性This antibody is expected to recognise isoforms 2( NP_001440.1) and 5 (NP_001153171.1).
  • アプリケーション適用あり: WB, IHC-Pmore details
  • 種交差性
    交差種: Mouse, Human
    交差が予測される動物種: Rat, Cow, Dog, Pig
  • 免疫原

    Synthetic peptide:

    (C)NKRFVFHQEQVY

    , corresponding to internal sequence amino acids 261-272 of Human FHL1

  • ポジティブ・コントロール
    • Lysate prepared from human muscle. Human testis tissue.

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • バッファーPreservative: 0.02% Sodium Azide
    Constituents: 0.5% BSA, Tris buffered saline. pH 7.3
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • 特記事項(精製)Purified by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
  • 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 ab23937 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
WB Use a concentration of 0.05 - 0.3 µg/ml. Detects a band of approximately 30 kDa (predicted molecular weight: 36 kDa).
IHC-P Use a concentration of 2 - 3 µg/ml. Perform heat mediated antigen retrieval via the pressure cooker method before commencing with IHC staining protocol.

ターゲット情報

  • 機能May have an involvement in muscle development or hypertrophy.
  • 組織特異性Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.
  • 関連疾患Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM) [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.
    Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies.
    Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases.
    Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (CO-RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].
  • 配列類似性Contains 3 LIM zinc-binding domains.
  • 発生段階Elevated levels during postnatal muscle growth.
  • 細胞内局在Cytoplasm; Cytoplasm. Nucleus and Nucleus. Cytoplasm > cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
  • Information by UniProt
  • 参照データベース
  • 別名
    • bA535K18.1 antibody
    • FHL 1 antibody
    • FHL 1B antibody
    • FHL-1 antibody
    • FHL1 antibody
    • FHL1 protein antibody
    • FHL1_HUMAN antibody
    • FHL1A antibody
    • FHL1B antibody
    • FLH1A antibody
    • Four and a half LIM domains 1 antibody
    • Four and a half LIM domains protein 1 antibody
    • Four and a half Lin11 Isl 1 and Mec 3 domains 1 antibody
    • four-and-a-half Lin11 antibody
    • Isl-1 and Mec-3 antibody
    • KYO T antibody
    • KYOT, mouse, homolog of antibody
    • LIM protein SLIMMER antibody
    • MGC111107 antibody
    • RAM14-1 antibody
    • RBMX1A antibody
    • RBMX1B antibody
    • RBP associated molecule 14-1 antibody
    • RP11-535K18.1 antibody
    • Skeletal muscle LIM protein 1 antibody
    • Skeletal muscle LIM-protein 1 antibody
    • SLIM 1 antibody
    • SLIM antibody
    • SLIM-1 antibody
    • SLIM1 antibody
    • SLIMMER antibody
    • XMPMA antibody
    see all

Anti-FHL1 antibody 画像

  • Anti-FHL1 antibody (ab23937) at 0.05 µg/ml + lysate prepared from human muscle (RIPA buffer) at 35 µg

    Predicted band size : 36 kDa
    Observed band size : 30 kDa (why is the actual band size different from the predicted?)
    Primary incubated for 1 hour. Detected by western blot using chemiluminescence.
  • ab23937 staining FHL1 in human testis tissue by Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections). Steamed antigen retrieval with citrate buffer pH 6 was performed. AP staining was used as the detection method. Primary antibody incubated at 2.5µg/ml.
    Image shows fibrous staining in the cytoplasm of developing sperm cells.

Anti-FHL1 antibody (ab23937) 使用論文

This product has been referenced in:
  • D'Arcy C  et al. X-linked Recessive Distal Myopathy With Hypertrophic Cardiomyopathy Caused by a Novel Mutation in the FHL1 Gene. J Child Neurol N/A:N/A (2014). IHC-Fr ; Human . Read more (PubMed: 25246303) »
  • D'Arcy CE  et al. Identification of FHL1 as a therapeutic target for Duchenne muscular dystrophy. Hum Mol Genet N/A:N/A (2013). Read more (PubMed: 24087791) »

See all 5 Publications for this product

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