Overview

  • Product name

  • Description

    Rabbit polyclonal to FHL1
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse, Human
    Predicted to work with: Rat, Sheep, Cow, Pig
  • Immunogen

    Recombinant fragment within Human FHL1 (internal sequence). The exact sequence is proprietary.
    Database link: Q13642

  • Positive control

    • IHC-P: Mouse hind brain, muscle and heart tissues; U-87 xenograft tissue. WB: Jurkat whole cell lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab229485 in the following tested applications.

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

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 36 kDa.
IHC-P 1/100 - 1/1000.

Target

  • Function

    May have an involvement in muscle development or hypertrophy.
  • Tissue specificity

    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.
  • Involvement in disease

    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].
  • Sequence similarities

    Contains 3 LIM zinc-binding domains.
  • Developmental stage

    Elevated levels during postnatal muscle growth.
  • Cellular localization

    Cytoplasm; Cytoplasm. Nucleus and Nucleus. Cytoplasm > cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
  • Information by UniProt
  • Database links

  • Alternative names

    • 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

Images

  • Anti-FHL1 antibody (ab229485) at 1/2000 dilution + Jurkat (human T cell leukemia cell line from peripheral blood) whole cell lysate at 30 µg

    Predicted band size: 36 kDa



    12% SDS-PAGE gel.

  • Paraffin-embedded mouse muscle tissue stained for FHL1 using ab229485 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded mouse hind brain tissue stained for FHL1 using ab229485 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded mouse heart tissue stained for FHL1 using ab229485 at 1/500 dilution in immunohistochemical analysis.

  • Paraffin-embedded U-87 xenograft tissue stained for FHL1 using ab229485 at 1/500 dilution in immunohistochemical analysis.

References

ab229485 has not yet been referenced specifically in any publications.

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