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, IHC-P, ICC/IFmore 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

    A region within synthetic peptide: VAKKCAGCKN PITGFGKGSS VVAYEGQSWH DYCFHCKKCS VNLANKRFVF, corresponding to C terminal amino acids 217-266 of Human FHL1

  • 阳性对照
    • WB: Fetal muscle lysate. IHC-P: Human kidney epithelial cells.

性能

相关产品

应用

Our Abpromise guarantee covers the use of ab49241 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 0.1 µg/ml. Detects a band of approximately 30 kDa (predicted molecular weight: 32 kDa). Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
IHC-P Use a concentration of 4 - 8 µg/ml.
ICC/IF Use a concentration of 1 µg/ml.

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 (ab49241) at 0.1 µg/ml + Fetal muscle lysate at 10 µg

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

    Predicted band size : 32 kDa
    Observed band size : 30 kDa (why is the actual band size different from the predicted?)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human kidney tissue labelling FHL1 with ab49241 at 4-8µg/ml. Arrows indicate positively labelled epithelial renal tubule cells. Magnification: 400X.

  • ICC/IF image of ab49241 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab49241, 1µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.

Anti-FHL1 antibody (ab49241)参考文献

This product has been referenced in:
  • Matulis CK & Mayo KE The LIM domain protein FHL2 interacts with the NR5A family of nuclear receptors and CREB to activate the inhibin-a subunit gene in ovarian granulosa cells. Mol Endocrinol 26:1278-90 (2012). Human . Read more (PubMed: 22734036) »
  • Feldkirchner S  et al. Patient-specific protein aggregates in myofibrillar myopathies: Laser microdissection and differential proteomics for identification of plaque components. Proteomics : (2012). Read more (PubMed: 23044792) »

See all 4 Publications for this product

Product Wall

Thank you for contacting us. I have some further information about the recommended dilution of this antibody in IHC-P. 4 µg/ml = 1:250 8 µg/ml = 1:125 I would start at 4 µg/ml and see whether the results are satisfactory, additional optimiz...

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