Overview

  • Product name
    Mouse Frataxin ELISA Kit, Fluorescent
    See all Frataxin kits
  • Detection method
    Fluorescent
  • Precision
    Intra-assay
    Sample n Mean SD CV%
    cell extract 3 1.3%
    Inter-assay
    Sample n Mean SD CV%
    cell extract 3 2%
  • Sample type
    Cell culture extracts, Tissue Extracts
  • Assay type
    Sandwich (quantitative)
  • Sensitivity
    4.8 pg/ml
  • Range
    7.8 pg/ml - 16000 pg/ml
  • Recovery

    Sample specific recovery
    Sample type Average % Range
    Serum 100 95% - 109%
    Fetal Bovine Serum 108 95% - 126%

  • Assay time
    1h 30m
  • Assay duration
    One step assay
  • Species reactivity
    Reacts with: Mouse
  • Product overview

    Frataxin in vitro CatchPoint SimpleStep ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of Frataxin protein in mouse cell and tissue extracts.


    This CatchPoint SimpleStep ELISA kit has been optimized for Molecular Devices Microplate Readers. Click here for a list of recommended Microplate Readers.
    If using a Molecular Devices’ plate reader supported by SoftMax® Pro software, a preconfigured protocol for these CatchPoint SimpleStep ELISA Kits is available with all the protocol and analysis settings at www.softmaxpro.org.


    The CatchPoint SimpleStep ELISA employs an affinity tag labeled capture antibody and a reporter conjugated detector antibody which immunocapture the sample analyte in solution. This entire complex (capture antibody/analyte/detector antibody) is in turn immobilized via immunoaffinity of an anti-tag antibody coating the well. To perform the assay, samples or standards are added to the wells, followed by the antibody mix. After incubation, the wells are washed to remove unbound material.  CatchPoint HRP Development Solution containing the Stoplight Red Substrate is added. During incubation, the substrate is catalyzed by HRP generating a fluorescent product. Signal is generated proportionally to the amount of bound analyte and the intensity is measured in a fluorescence plater reader at 530/570/590 nm Excitation/Cutoff/Emission.

  • Notes

    Mouse Frataxin is a 23 kDa nuclear-encoded mitochondrial protein. The gene is localized on chromosome 9 and is highly conserved during evolution. The gene is expressed in every cell, although in varying levels in different tissues and during development. The specific function of frataxin is still unknown, but it has been shown to play a role in iron metabolism. Studies have demonstrated that the deletion of the frataxin gene in yeast results in iron accumulation in mitochondria and loss of respiration. Recombinant frataxin has been shown to bind iron in vitro, and increased mitochondrial iron levels have been observed in patients with Friedreich's Ataxia (FRDA). FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. 

  • Tested applications
    Suitable for: Sandwich ELISAmore details
  • Platform
    Pre-coated microplate (12 x 8 well strips)

Properties

  • Storage instructions
    Store at +4°C. Please refer to protocols.
  • Components 1 x 96 tests
    100X Stoplight Red Substrate 1 x 120µl
    10X Mouse Frataxin Capture Antibody 1 x 600µl
    10X Mouse Frataxin Detector Antibody 1 x 600µl
    10X Wash Buffer PT (ab206977) 1 x 20ml
    500X Hydrogen Peroxide (H2O2, 3%) 1 x 50µl
    50X Cell Extraction Enhancer Solution 1 x 1ml
    5X Cell Extraction Buffer PTR (ab193970) 1 x 10ml
    Antibody Diluent 5BI 1 x 6ml
    Mouse Frataxin Lyophilized Recombinant Protein 2 vials
    Plate Seals 1 unit
    Sample Diluent NS 1 x 12ml
    SimpleStep Pre-Coated Black 96-Well Microplate 1 unit
    Stoplight Red Substrate Buffer 1 x 12ml
  • Research areas
  • 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.
  • 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

Applications

Our Abpromise guarantee covers the use of ab229424 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
Sandwich ELISA Use at an assay dependent concentration.

Images

  • ELISA Protocol Summary
  • The Frataxin standard curve was prepared as described in Section 10.

  • NIH‑3T3, RAW264.7 and, mouse liver extracts were prepared as described previously and interpolated values corrected by dilution factor (mean +/- SD) are graphed.

  • Interpolated values of mouse Frataxin protein are plotted for the indicated cell and tissue extract based on an extract load of 1,250 ng/mL.

Protocols

References

ab229424 has not yet been referenced specifically in any publications.

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