Overview

  • Product nameAnti-FHL1 antibody
    See all FHL1 primary antibodies
  • Description
    Mouse monoclonal to FHL1
  • Tested applicationsSuitable for: WB, IHC-P, Flow Cytmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment: DGHHCCLKCF DKFCANTCVE CRKPIGADSK EVHYKNRFWH DTCFRCAKCL HPLANETFVA KDNKILCNKC TTREDSPKCK GCFKAIVAGD QNVEYKGT, corresponding to amino acids 23-121 of Human FHL1

Properties

Applications

Our Abpromise guarantee covers the use of ab58067 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 Use a concentration of 1 - 5 µg/ml.
IHC-P Use at an assay dependent concentration. PubMed: 24114807
Flow Cyt Use 0.5µg for 106 cells. ab170191-Mouse monoclonal IgG2a, is suitable for use as an isotype control with this antibody.

Target

  • FunctionMay have an involvement in muscle development or hypertrophy.
  • Tissue specificityIsoform 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 diseaseDefects 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 similaritiesContains 3 LIM zinc-binding domains.
  • Developmental stageElevated levels during postnatal muscle growth.
  • Cellular localizationCytoplasm; 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

Anti-FHL1 antibody images

  • FHL1 antibody (ab58067) at 1ug/lane + Raw cell lysate at 25ug/lane.
  • Overlay histogram showing SH-SY5Y cells stained with ab58067 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab58067, 0.5µg/1x106 cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 2µg/1x106 cells) used under the same conditions. Acquisition of >5,000 events was performed.

References for Anti-FHL1 antibody (ab58067)

This product has been referenced in:
  • Christodoulou DC  et al. 5'RNA-Seq identifies Fhl1 as a genetic modifier in cardiomyopathy. J Clin Invest 124:1364-70 (2014). WB ; Mouse . Read more (PubMed: 24509080) »
  • Domenighetti AA  et al. Loss of FHL1 induces an age-dependent skeletal muscle myopathy associated with myofibrillar and intermyofibrillar disorganization in mice. Hum Mol Genet N/A:N/A (2013). Read more (PubMed: 23975679) »

See all 5 Publications for this product

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