Recombinant人FHL1 protein (ab112286)



  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionQ13642
    • 种属Human
    • 分子量36 kDa
    • 氨基酸23 to 120
    • 标签GST tag N-Terminus


Our Abpromise guarantee covers the use of ab112286 in the following tested applications.

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

  • 应用


    Western blot


  • 形式Liquid
  • Concentration information loading...


  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.79% Tris HCl, 0.31% Glutathione


  • 别名
    • bA535K18.1
    • FHL 1
    • FHL 1B
    • FHL-1
    • FHL1
    • FHL1 protein
    • FHL1_HUMAN
    • FHL1A
    • FHL1B
    • FLH1A
    • Four and a half LIM domains 1
    • Four and a half LIM domains protein 1
    • Four and a half Lin11 Isl 1 and Mec 3 domains 1
    • four-and-a-half Lin11
    • Isl-1 and Mec-3
    • KYO T
    • KYOT, mouse, homolog of
    • LIM protein SLIMMER
    • MGC111107
    • RAM14-1
    • RBMX1A
    • RBMX1B
    • RBP associated molecule 14-1
    • RP11-535K18.1
    • Skeletal muscle LIM protein 1
    • Skeletal muscle LIM-protein 1
    • SLIM
    • SLIM 1
    • SLIM-1
    • SLIM1
    • XMPMA
    see all
  • 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

Recombinant Human FHL1 protein 图像

  • ab112286 analysed on a 12.5% SDS-PAGE stained with Coomassie Blue.

Recombinant Human FHL1 protein (ab112286)参考文献

ab112286 has not yet been referenced specifically in any publications.

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