Overview

  • Product name
    Anti-Fukutin antibody [EPR7913]
    See all Fukutin primary antibodies
  • Description
    Rabbit monoclonal [EPR7913] to Fukutin
  • Host species
    Rabbit
  • Tested applications
    Suitable for: ICC/IF, WB, IHC-Pmore details
    Unsuitable for: Flow Cyt or IP
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human Fukutin aa 400-500. The exact sequence is proprietary.

  • Positive control
    • HeLa, A549, fetal heart, and BxPC-3 lysates; Human kidney tissue.
  • General notes

     

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab131280 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
ICC/IF 1/250 - 1/500.
WB 1/1000 - 1/10000. Predicted molecular weight: 53 kDa.
IHC-P 1/100 - 1/250. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
  • Application notes
    Is unsuitable for Flow Cyt or IP.
  • Target

    • Function
      May be a glycosyltransferase which participates in glycosylation of alpha-dystroglycan/DAG1. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development.
    • Tissue specificity
      Widely expressed with highest expression in brain, heart, pancreas and skeletal muscle. Expressed at similar levels in control fetal and adult brain, but is much reduced in Fukuyama-type congenital dystrophy (FCMD) brains. Expressed in migrating neurons, including Cajar-Retzius cells and adult cortical neurons, as well as hippocampal pyramidal cells and cerebellar Purkinje cells. No expression observed in the glia limitans, the subpial astrocytes (which contribute to basement membrane formation) or other glial cells. In the FCMD brain, neurons in regions with no dysplasia show fair expression, whereas transcripts are nearly undetectable in the overmigrated dysplastic region.
    • Involvement in disease
      Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies type A4 (MDDGA4) [MIM:253800]; also called congenital muscular dystrophy Fukuyama type (FCMD) or Walker-Warburg syndrome FKTN-related. MDDGA4 is an autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies. Patients suffer from generalized skeletal muscle weakness and hypotonia from early infancy, mental retardation and seizures. Occasional features include optic atrophy, retinal detachment, cardiomyopathy.
      Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy congenital without mental retardation type B4 (MDDGB4) [MIM:613152]. An autosomal recessive disorder characterized by congenital muscular dystrophy and evidence of dystroglycanopathy. Features included increased serum creatine kinase, generalized weakness, mild white matter changes on brain MRI in some cases, and absence of mental retardation.
      Defects in FKTN are the cause of muscular dystrophy-dystroglycanopathy limb-girdle type C4 (MDDGC4) [MIM:611588]. MDDGC4 is an autosomal recessive degenerative myopathy characterized by progressive weakness of the pelvic and shoulder girdle muscles and elevated serum creatine kinase. The severity of the disease depends on age at onset which may vary from early to late childhood or even adulthood. MDDGC4 is a novel form of LGMD2 and has no brain involvement and a remarkable clinical response to corticosteroids.
      Defects in FKTN are the cause of cardiomyopathy dilated type 1X (CMD1X) [MIM:611615]; also called dilated cardiomyopathy with mild or no proximal muscle weakness. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
    • Sequence similarities
      Belongs to the licD transferase family.
    • Cellular localization
      Golgi apparatus membrane.
    • Information by UniProt
    • Database links
    • Alternative names
      • CMD1X antibody
      • FCMD antibody
      • FCMD gene antibody
      • FKTN antibody
      • FKTN_HUMAN antibody
      • Fukutin antibody
      • Fukuyama type congenital muscular dystrophy protein antibody
      • Fukuyama-type congenital muscular dystrophy protein antibody
      • LGMD2M antibody
      • MDDGA4 antibody
      • MDDGB4 antibody
      • MDDGC4 antibody
      • MGC126857 antibody
      • MGC134944 antibody
      • MGC134945 antibody
      • MGC138243 antibody
      • OTTHUMP00000021841 antibody
      • patient fukutin antibody
      see all

    Images

    • All lanes : Anti-Fukutin antibody [EPR7913] (ab131280) at 1/1000 dilution

      Lane 1 : HeLa lysate
      Lane 2 : A549 lysate
      Lane 3 : Fetal heart lysate
      Lane 4 : BxPC-3 lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size: 53 kDa

    • Immunohistochemical analysis of paraffin-embedded Human kidney tissue labelling Fukutin with ab131280 at 1/100 dilution.
    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    References

    This product has been referenced in:
    See 1 Publication for this product

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