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    human-frataxin-matched-antibody-pair-kit-ab212062.pdf

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Human Frataxin Matched Antibody Pair Kit (ab212062)

  • Datasheet
  • SDS
  • Protocol Booklet
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Human Frataxin standard curve.

    Key features and details

    • Unlabeled capture antibody, biotin-labeled detection antibody and calibrated protein standard
    • For economical ELISA and ELISA-based assay development
    • Reacts with: Human
    • Range: 39 pg/ml - 2500 pg/ml

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    Protein
    Product image
    Recombinant Human Frataxin protein (ab110353)
    Pair
    ELISA Accessory Pack (ab210905)

    View more associated products

    Overview

    • Product name

      Human Frataxin Matched Antibody Pair Kit
      See all Frataxin kits
    • Detection method

      Colorimetric
    • Assay type

      ELISA set
    • Sensitivity

      8.4 pg/ml
    • Range

      39 pg/ml - 2500 pg/ml
    • Species reactivity

      Reacts with: Human
    • Product overview

      Human Frataxin Matched Antibody Pair Kits include a capture and a biotinylated detector antibody pair, along with a calibrated protein standard, suitable for sandwich ELISA. The Matched Antibody Pair Kit can be used to quantify native and recombinant human Frataxin.


      Optimization of the kit reagents to sample type, immunoassay format or instrumentation may be required. Guidelines for use of this kit in a standard 96-well microplate sandwich ELISA using HRP/TMB system of colorimetric detection is described in this assay procedure for the purposes of quantification.


      Protocol information and tips on the use of the Matched Antibody Pair kits for sandwich ELISA can be found on our website. An accessory pack can be purchased which includes buffer reagents required to perform 10 x 96-well plate sandwich ELISAs (ab210905).


      For additional information on the performance of the antibody pair used in this kit, please see our equivalent SimpleStep ELISA kit ab176112. Please note that while the antibody pair is the same provided in the corresponding SimpleStep ELISA Kit, due to differences in their formulation, this antibody pair cannot be used with the consumables provided with our SimpleStep ELISA Kits.

    • Tested applications

      Suitable for: ELISA, IAmore details
    • Platform

      Reagents

    Properties

    • Storage instructions

      Store at -20°C. Please refer to protocols.
    • Components 10 x 96 tests 5 x 96 tests
      Human Frataxin Capture Antibody 2 x 50µg 1 x 50µg
      Human Frataxin Detector Antibody 2 x 12.5µg 1 x 12.5µg
      Human Frataxin Lyophilized Protein 2 vials 1 vial
    • Research areas

      • Tags & Cell Markers
      • Subcellular Markers
      • Organelles
      • Mitochondria
      • Signal Transduction
      • Metabolism
      • Mitochondrial
      • Metabolism
      • Pathways and Processes
      • Mitochondrial Metabolism
      • Mitochondrial markers
    • Function

      Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.
    • Tissue specificity

      Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
    • Involvement in disease

      Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region.
    • Sequence similarities

      Belongs to the frataxin family.
    • Post-translational
      modifications

      Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure.
    • Cellular localization

      Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
    • Target information above from: UniProt accession Q16595 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Alternative names

      • CyaY
      • d-FXN
      • FA
      • FARR
      • Frataxin mature form
      • Frataxin(81-210)
      • FRDA
      • FRDA_HUMAN
      • Friedreich ataxia protein
      • Fxn
      • i-FXN
      • m56-FXN
      • m78-FXN
      • m81-FXN
      • MGC57199
      • X25
      see all
    • Database links

      • Entrez Gene: 2395 Human
      • Omim: 606829 Human
      • SwissProt: Q16595 Human
      • Unigene: 20685 Human

      Associated products

      • Related Products

        • ELISA Accessory Pack (ab210905)

      Applications

      Our Abpromise guarantee covers the use of ab212062 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
      ELISA Use at an assay dependent concentration.
      IA Use at an assay dependent concentration.

      Images

      • Human Frataxin standard curve.
        Human Frataxin standard curve.

        Standard calibration curve. Background subtracted values are graphed

      Protocols

      • Protocol Booklet

      Click here to view the general protocols

      Datasheets and documents

      • Datasheet
      • SDS
    • References (0)

      Publishing research using ab212062? Please let us know so that we can cite the reference in this datasheet.

      ab212062 has not yet been referenced specifically in any publications.

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