概述

  • 产品名称Anti-FHL1抗体
    参阅全部 FHL1 一抗
  • 描述
    兔多克隆抗体to FHL1
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Sheep, Rabbit, Horse, Guinea pig, Cow, Cat, Dog, Pig
  • 免疫原

    Synthetic peptide from within the N terminal amino acids 51-100: SKEVHYKNRF WHDTCFRCAK CLHPLANETF VAKDNKILCN KCTTREDSPK, of Human FHL1 (NP_001440)

  • 阳性对照
    • Human fetal brain lysate

性能

应用

Our Abpromise guarantee covers the use of ab83571 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. Detects a band of approximately 32 kDa (predicted molecular weight: 36 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay: 1:62500.

靶标

  • 功能May have an involvement in muscle development or hypertrophy.
  • 组织特异性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.
  • 疾病相关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].
  • 序列相似性Contains 3 LIM zinc-binding domains.
  • 发展阶段Elevated levels during postnatal muscle growth.
  • 细胞定位Cytoplasm; Cytoplasm. Nucleus and Nucleus. Cytoplasm > cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 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 图像

  • Anti-FHL1 antibody (ab83571) at 1 µg/ml (in 5% skim milk/ PBS) + human fetal brain lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 36 kDa
    Observed band size : 32 kDa (why is the actual band size different from the predicted?)

Anti-FHL1 antibody (ab83571)参考文献

ab83571 has not yet been referenced specifically in any publications.

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