Human Frataxin ELISA Kit is a single-wash 90-min Simplestep used to quantify Human Frataxin with a sensitivity of 2.1 pg/mL. The assay uses a simple mix-wash-read protocol with just one incubation and one wash step.
- Colorimetric Sandwich ELISA - 450 nm readout : works on any standard plate reader
- Different formats for different needs: 384-well plate for higher throughput
- Design your own immunoassay: we also offer the conjugation-ready antibody pair
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- Disease models & mechanisms 16:2023Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.Applications:Unspecified applicationReactive species:Unspecified reactive speciesNicole M Sayles,Jill S Napierala,Josef Anrather,Nadège Diedhiou,Jixue Li,Marek Napierala,Hélène Puccio,Giovanni ManfrediPubMed 37691621
- Genes 14:2023Finding an Appropriate Mouse Model to Study the Impact of a Treatment for Friedreich Ataxia on the Behavioral Phenotype.Applications:Unspecified applicationReactive species:Unspecified reactive speciesCamille Bouchard,Catherine Gérard,Solange Gni-Fiene Yanyabé,Nathalie Majeau,Malek Aloui,Gabrielle Buisson,Pouiré Yameogo,Vanessa Couture,Jacques P TremblayPubMed 37628705
- Molecular therapy. Methods & clinical development 19:120-1382020High Levels of Frataxin Overexpression Lead to Mitochondrial and Cardiac Toxicity in Mouse Models.Applications:Unspecified applicationReactive species:Unspecified reactive speciesBrahim Belbellaa et. al.PubMed 33209958
- Molecular therapy : the journal of the American So 26:1940-19522018Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia.Applications:Unspecified applicationReactive species:Unspecified reactive speciesFrançoise Piguet et. al.PubMed 29853274
- Bioorganic & medicinal chemistry 26:3359-33692018Lipophilic methylene blue analogues enhance mitochondrial function and increase frataxin levels in a cellular model of Friedreich's ataxia.Applications:Unspecified applicationReactive species:Unspecified reactive speciesOmar M Khdour et. al.PubMed 29773347
- Journal of cellular and molecular medicine 22:834-8482017Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment.Applications:Unspecified applicationReactive species:Unspecified reactive speciesElena Britti,Fabien Delaspre,Anat Feldman,Melissa Osborne,Hagar Greif,Jordi Tamarit,Joaquim RosPubMed 28980774
- Annals of neurology 81:212-2262016Cytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse model.Applications:sELISAReactive species:HumanKevin C Kemp,Nadia Cerminara,Kelly Hares,Juliana Redondo,Amelia J Cook,Harry R Haynes,Bronwen R Burton,Mark Pook,Richard Apps,Neil J Scolding,Alastair WilkinsPubMed 28009062
Key facts
- Detection method
- Colorimetric
- Sample types
- Cell culture extracts, Tissue Extracts
- Assay type
- Sandwich (quantitative)
- Reactive species
- Human
- Range
- 9.88 - 800 pg/mL
- Assay time
- 1h 30m
- Sensitivity
- = 2.1 pg/mL
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Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application sELISA | Reactivity Reacts | Dilution info - | Notes - |
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Target data
Function
Frataxin mature form. Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly (PubMed:12785837, PubMed:24971490). Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release (PubMed:24971490). Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (PubMed:29576242). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity). 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 (PubMed:15641778). 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 (PubMed:11823441, PubMed:12755598). May function as an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation (PubMed:15247478). May play a role as a high affinity iron binding partner for FECH that is capable of both delivering iron to ferrochelatase and mediating the terminal step in mitochondrial heme biosynthesis (PubMed:15123683, PubMed:16239244). Extramitochondrial frataxin. Modulates the RNA-binding activity of ACO1 (PubMed:20053667). May be involved in the cytoplasmic iron-sulfur protein biogenesis (PubMed:16091420). May contribute to oxidative stress resistance and overall cell survival (PubMed:16608849).
Alternative names
FRDA, X25, FXN, Friedreich ataxia protein, Fxn
What's included?
Recommended products
Human Frataxin ELISA Kit is a single-wash 90-min Simplestep used to quantify Human Frataxin with a sensitivity of 2.1 pg/mL. The assay uses a simple mix-wash-read protocol with just one incubation and one wash step.
- Colorimetric Sandwich ELISA - 450 nm readout : works on any standard plate reader
- Different formats for different needs: 384-well plate for higher throughput
- Design your own immunoassay: we also offer the conjugation-ready antibody pair
Key facts
- Detection method
- Colorimetric
- Sample types
- Cell culture extracts, Tissue Extracts
- Assay type
- Sandwich (quantitative)
- Reactive species
- Human
- Range
- 9.88 - 800 pg/mL
- Assay time
- 1h 30m
- Assay Platform
- Microplate
- Sensitivity
- = 2.1 pg/mL
Precision
Intra assay
| Sample | n | C.V. |
|---|---|---|
Sample HeLa | n 9 | C.V. 2.3 |
Inter assay
| Sample | n | C.V. |
|---|---|---|
Sample HeLa | n 3 | C.V. 6.3 |
Recovery
Sample specific recovery
| Sample type | Average % | Range |
|---|---|---|
Sample type Cell culture media | Average % = 97 | Range 93 - 101 % |
Sample type EDTA Plasma | Average % = 89 | Range 88 - 92 % |
Sample type Serum | Average % = 92 | Range 86 - 98 % |
Storage
- Shipped at conditions
- Blue Ice
- Appropriate short-term storage conditions
- +4°C
- Appropriate long-term storage conditions
- +4°C
- Storage information
- +4°C
Notes
Human Frataxin ELISA Kit ab176112 is a rapid single-wash 90-min ELISA kit to measure Human Frataxin in cell culture extracts and tissue extracts. This SimpleStep ELISA® sensitivity is 2.1 pg/mL.
How the assay works
Human Frataxin SimpleStep ELISA® 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.
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.
Human Frataxin ELISA Kit ab176112 protocol summary:
1. Mix: add samples/standards to the wells together with the capture and detector antibody cocktail. Incubate for 1 hr at room temperature
2. Wash
3. Add TMB development solution - incubate for 10 min
4. Add stop solution
5. Read the results on a plate reader at 450nm
How other researchers are using Human Frataxin ELISA Kit ab176112
Human Frataxin ELISA Kit ab176112 has been used to study effects and mechanism of Friedreich ataxia including cardiac mitochondrial stress(1), treatment with trans-activator of transcription peptides(2), and treatment with cytokine therapy (3).
References:
(1)Giovanni Manfredi et al. 2023, PMID: 37691621,(2)Joaquim Ros et al. 2018, PMID: 28980774,
(3)Alastair Wilkins et al. 2017, PMID: 28009062
Save your precious samples
Our 384-well Human Frataxin ELISA Kit only requires a maxmum of 12.5µL
Design your own immunoassay
We offer the antibody pair used in this kit in a BSA and Azide-free format, ready for conjugation
- Human Frataxin Antibody Pair - BSA and Azide free Human Frataxin Antibody Pair - BSA and Azide free ab256593
- Anti-Frataxin antibody [30-17A11AC7] - BSA and Azide free (Capture) Anti-Frataxin antibody [30-17A11AC7] - BSA and Azide free (Capture) ab259433
- Anti-Frataxin antibody [30-18A5DB1] - BSA and Azide free (Detector) Anti-Frataxin antibody [30-18A5DB1] - BSA and Azide free (Detector) ab259434
Related and recommended products
Human Frataxin ELISA Kit ab176112 is commonly used to study Friedreich's ataxia, a neurodegenerative disease caused by frataxin mutations leading to oxidative stress.
Other products to measure frataxin include:
- Mouse Frataxin ELISA Kit Mouse Frataxin ELISA Kit ab199078
- Anti-Frataxin antibody [17A11] Anti-Frataxin antibody [17A11] ab113691
- Recombinant Human Frataxin protein Recombinant Human Frataxin protein ab95502
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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10 product images
ELISA - Human Frataxin ELISA Kit (ab176112)
Titration of HeLa cell extract within the working range of the assay.
Background subtracted data from duplicate measurements are plotted.
ELISA - Human Frataxin ELISA Kit (ab176112)
Quantification of Frataxin expression in different cell lines.
Interpolated values of Frataxin are plotted for the indicated cell lines based on a extract load of 10 μg/mL.
This image is courtesy of an anonymous customer reviewSandwich ELISA - Human Frataxin ELISA Kit (ab176112)
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.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Linearity of dilution for Recombinant Frataxin.
Recombinant Frataxin was measured in biological samples in a 2-fold dilution series
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Example of Frataxin standard curve.
Background-subtracted data values (mean +/- SD) are graphed.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
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.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Quantification of Frataxin expression in different human patient samples.
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.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Interpolated concentration of native Frataxin was measured in duplicate at different sample concentrations in 96-well and 384-well plates. Undiluted samples are 50 µg/mL HeLa cell lysate. The interpolated dilution factor corrected values are plotted (mean +/- SD, n=2). Sample dilutions are made in 1X Cell Extraction Buffer PTR.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Example of Human Frataxin standard curve in 96-well and 384-well plate. Background-subtracted data values (mean +/- SD) are graphed.
Sandwich ELISA - Human Frataxin ELISA Kit (ab176112)
Raw data value for example of frataxin standard curve.
Raw data values are shown in the table
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