Human Frataxin ELISA Kit (ab176112)
Key features and details
- One-wash 90 minute protocol
- Sensitivity: 2.1 pg/ml
- Range: 9.88 pg/ml - 800 pg/ml
- Sample type: Cell culture extracts, Tissue Extracts
- Detection method: Colorimetric
- Assay type: Sandwich (quantitative)
- Reacts with: Human
Product nameHuman Frataxin ELISA Kit
See all Frataxin kits
Intra-assay Sample n Mean SD CV% HeLa 9 2.3% Inter-assay Sample n Mean SD CV% HeLa 3 6.3%
Sample typeCell culture extracts, Tissue Extracts
Assay typeSandwich (quantitative)
Range9.88 pg/ml - 800 pg/ml
Sample specific recovery Sample type Average % Range Serum 92 86% - 98% Cell culture media 97 93% - 101% EDTA Plasma 89 88% - 92%
Assay time1h 30m
Assay durationOne step assay
Species reactivityReacts with: Human
Human Frataxin ELISA kit (ab176112) is a single-wash 90 min sandwich ELISA designed for the quantitative measurement of Frataxin protein in human cell culture and tissue extracts. It uses our proprietary SimpleStep ELISA® technology. Quantitate human Frataxin with 1.1 pg/ml sensitivity.
SimpleStep ELISA® technology employs capture antibodies conjugated to an affinity tag that is recognized by the monoclonal antibody used to coat our SimpleStep ELISA® plates. This approach to sandwich ELISA allows the formation of the antibody-analyte sandwich complex in a single step, significantly reducing assay time. See the SimpleStep ELISA® protocol summary in the image section for further details. Our SimpleStep ELISA® technology provides several benefits:
-Single-wash protocol reduces assay time to 90 minutes or less
-High sensitivity, specificity and reproducibility from superior antibodies
-Fully validated in biological samples
-96-wells plate breakable into 12 x 8 wells strips
A 384-well SimpleStep ELISA® microplate (ab203359) is available to use as an alternative to the 96-well microplate provided with SimpeStep ELISA® kits.
ASSAY SPECIFICITY This kit recognizes both native and recombinant human Frataxin protein in cell and tissue extract samples only. Serum, plasma, saliva, and urine samples have not been tested with this kit.
Frataxin is a 17 kDa nuclear-encoded mitochondrial protein. In humans 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 human 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.
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Storage instructionsStore at +4°C. Please refer to protocols.
Components 1 x 96 tests 10X Human Frataxin Capture Antibody 1 x 600µl 10X Human Frataxin Detector Antibody 1 x 600µl 10X Wash Buffer PT (ab206977) 1 x 20ml 50X Cell Extraction Enhancer Solution (ab193971) 1 x 1ml 5X Cell Extraction Buffer PTR (ab193970) 1 x 10ml Antibody Diluent 4BI 1 x 6ml Human Frataxin Lyophilized Recombinant Protein 2 vials Plate Seals 1 unit Sample Diluent NS (ab193972) 1 x 12ml SimpleStep Pre-Coated 96-Well Microplate (ab206978) 1 unit Stop Solution 1 x 12ml TMB Development Solution 1 x 12ml
RelevanceFunction: Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ 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 Fe2+ to Fe3+; 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.
Cellular localizationCytoplasmic and Mitochondrial
- Entrez Gene: 2395 Human
- Omim: 606829 Human
- SwissProt: Q16595 Human
SimpleStep ELISA technology allows the formation of the antibody-antigen complex in one single step, reducing assay time to 90 minutes. Add samples or standards and antibody mix to wells all at once, incubate, wash, and add your final substrate. See protocol for a detailed step-by-step guide.
Raw data values are shown in the table
Background-subtracted data values (mean +/- SD) are graphed.
Background subtracted data from duplicate measurements are plotted.
Interpolated values of Frataxin are plotted for the indicated cell lines based on a extract load of 10 µg/mL.
Transformed B lymphocyte cells from Friedreich's Ataxia (FRDA) samples, due to a stable homozygous GAA repeat insertion, are compared to heterozygous carrier B lymphocyte cells and control B lymphocyte cells. B lymphocyte cell extracts were analyzed across a 7-point titration (0.1-100 µg/mL) and frataxin levels were interpolated from the standard curve. Average interpolated values of Frataxin are plotted.
Standard curve generated using Abcam's Frataxin Human SimpleStep ELISA™ Kit (ab176112) with human brain tissue homogenate mixed in different proportions with mouse brain tissue homogenate. ab176112 did not cross-react with mouse frataxin. Both kinetic and endpoint readouts were used sequentially for a larger dynamic range. ab110353, 12ng human frataxin full length protein was also used to extended the standard curve to 12ng/ml.
Recombinant Frataxin was measured in biological samples in a 2-fold dilution series
Datasheets and documents
ab176112 has been referenced in 5 publications.
- Belbellaa B et al. High Levels of Frataxin Overexpression Lead to Mitochondrial and Cardiac Toxicity in Mouse Models. Mol Ther Methods Clin Dev 19:120-138 (2020). PubMed: 33209958
- Britti E et al. Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment. J Cell Mol Med 22:834-848 (2018). PubMed: 28980774
- Khdour OM et al. Lipophilic methylene blue analogues enhance mitochondrial function and increase frataxin levels in a cellular model of Friedreich's ataxia. Bioorg Med Chem 26:3359-3369 (2018). PubMed: 29773347
- Piguet F et al. Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia. Mol Ther 26:1940-1952 (2018). PubMed: 29853274
- Kemp KC et al. Cytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse model. Ann Neurol N/A:N/A (2016). Sandwich ELISA ; Human . PubMed: 28009062