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    fukutin-antibody-epr7913-ab131280.pdf

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RecombinantRabMAb

Recombinant Anti-Fukutin antibody [EPR7913] (ab131280)

  • Datasheet
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Western blot - Anti-Fukutin antibody [EPR7913] (ab131280)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Fukutin antibody [EPR7913] (ab131280)
  • OI-RD Scanning - Anti-Fukutin antibody [EPR7913] (ab131280)
  • Anti-Fukutin antibody [EPR7913] (ab131280)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • Rabbit monoclonal [EPR7913] to Fukutin
  • Suitable for: WB, IHC-P
  • Reacts with: Human

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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: WB, IHC-Pmore details
    Unsuitable for: Flow Cyt or IP
  • Species reactivity

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

    Synthetic peptide. This information is proprietary to Abcam and/or its suppliers.

  • Positive control

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

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

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

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • Dissociation constant (KD)

    KD = 5.00 x 10 -11 M
    Learn more about KD
  • Storage buffer

    pH: 7.20
    Preservative: 0.01% Sodium azide
    Constituents: 9% PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA, 50% Tissue culture supernatant
  • Concentration information loading...
  • Purity

    Protein A purified
  • Clonality

    Monoclonal
  • Clone number

    EPR7913
  • Isotype

    IgG
  • Research areas

    • Tags & Cell Markers
    • Subcellular Markers
    • Organelles
    • Golgi
    • Developmental Biology
    • Organogenesis
    • Skeletal development
    • Muscle
    • Developmental Biology
    • Organogenesis
    • Nervous system development

Associated products

  • Alternative Versions

    • Anti-Fukutin antibody [EPR7913] - BSA and Azide free (ab248403)
  • Isotype control

    • Rabbit IgG, monoclonal [EPR25A] - Isotype Control (ab172730)
  • Positive Controls

    • HeLa whole cell lysate (ab29545)
    • A549 whole cell lysate (ab7910)

Applications

The Abpromise guarantee

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
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.
Notes
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.
  • Target information above from: UniProt accession O75072 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links

    • Entrez Gene: 2218 Human
    • Entrez Gene: 246179 Mouse
    • Entrez Gene: 362520 Rat
    • Omim: 607440 Human
    • SwissProt: O75072 Human
    • SwissProt: Q8R507 Mouse
    • Unigene: 55777 Human
    • Unigene: 247210 Mouse
    • 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

    • Western blot - Anti-Fukutin antibody [EPR7913] (ab131280)
      Western blot - Anti-Fukutin antibody [EPR7913] (ab131280)
      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

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Fukutin antibody [EPR7913] (ab131280)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Fukutin antibody [EPR7913] (ab131280)

      Immunohistochemical analysis of paraffin-embedded Human kidney tissue labelling Fukutin with ab131280 at 1/100 dilution.

      Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

    • OI-RD Scanning - Anti-Fukutin antibody [EPR7913] (ab131280)
      OI-RD Scanning - Anti-Fukutin antibody [EPR7913] (ab131280)
      Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD
    • Anti-Fukutin antibody [EPR7913] (ab131280)
      Anti-Fukutin antibody [EPR7913] (ab131280)

    Protocols

    • Western blot protocols
    • Immunohistochemistry protocols

    Click here to view the general protocols

    Datasheets and documents

    • Datasheet download

      Download

    References (3)

    Publishing research using ab131280? Please let us know so that we can cite the reference in this datasheet.

    ab131280 has been referenced in 3 publications.

    • Pham TT  et al. Characteristic expression of fukutin in gastric cancer among atomic bomb survivors. Oncol Lett 13:937-941 (2017). PubMed: 28356981
    • Gerin I  et al. ISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto a-dystroglycan. Nat Commun 7:11534 (2016). PubMed: 27194101
    • Oo HZ  et al. Fukutin, identified by the Escherichia coli ampicillin secretion trap (CAST) method, participates in tumor progression in gastric cancer. Gastric Cancer 19:443-452 (2016). PubMed: 26223471

    Customer reviews and Q&As

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