Mouse Frataxin ELISA Kit is a single-wash 90-min Simplestep used to quantify Mouse Frataxin with a sensitivity of 5 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: 10x96 plates for bulk orders and 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
- 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
Key facts
- Detection method
- Colorimetric
- Sample types
- Cell culture extracts, Tissue Extracts
- Assay type
- Sandwich (quantitative)
- Reactive species
- Mouse
- Range
- 31.25 - 2000 pg/mL
- Assay time
- 1h 30m
- Sensitivity
- = 5 pg/mL
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:19805308, PubMed:25597503). 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:25597503). Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (By similarity). 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. 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. May function as an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation. 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 (By similarity). Extramitochondrial frataxin. Modulates the RNA-binding activity of ACO1. May be involved in the cytoplasmic iron-sulfur protein biogenesis. May contribute to oxidative stress resistance and overall cell survival.
Alternative names
Frda, Fxn
What's included?
Recommended products
Mouse Frataxin ELISA Kit is a single-wash 90-min Simplestep used to quantify Mouse Frataxin with a sensitivity of 5 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: 10x96 plates for bulk orders and 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
- Mouse
- Range
- 31.25 - 2000 pg/mL
- Assay time
- 1h 30m
- Assay Platform
- Microplate (12 x 8 well strips)
- Sensitivity
- = 5 pg/mL
Precision
Intra assay
| Sample | n | C.V. |
|---|---|---|
Sample NIH 3T3 | n 3 | C.V. 1.3 |
Inter assay
| Sample | n | C.V. |
|---|---|---|
Sample NIH 3T3 | n 3 | C.V. 2 |
Recovery
Sample specific recovery
| Sample type | Average % | Range |
|---|---|---|
Sample type Fetal Bovine Serum | Average % = 101 | Range 99 - 103 % |
Sample type Serum | Average % = 100 | Range 95 - 109 % |
Sample type serum free media | Average % = 101 | Range 99 - 103 % |
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
Mouse Frataxin ELISA Kit (ab199078) is a single-wash 90 min sandwich ELISA designed for the quantitative measurement of Frataxin protein in cell culture extracts and tissue extracts. It uses our proprietary SimpleStep ELISA® technology. Quantitate Mouse Frataxin with 5 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 (Pre-coated 384 well Microplate SimpleStep ELISA® ab203359) is available to use as an alternative to the 96-well microplate provided with SimpleStep ELISA® kits.
ASSAY SPECIFICITY
This kit recognizes both native and recombinant mouse Frataxin protein in cell and tissue extract samples only.
Whole blood, saliva, urine, serum and plasma samples have not been tested with this kit.
SPECIES REACTIVITY
This kit recognizes mouse Frataxin protein.
Human reactivity was determined to be 48%, by measuring 310 pg/mL human recombinant protein and comparing the raw O.D. values to the mouse standard protein raw O.D. values assayed at the same concentration, and expressing the ratio as a percentage of human protein reactivity to mouse protein reactivity within the assay.
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.
Abcam has not and does not intend to apply for the REACH Authorisation of customers' uses of products that contain European Authorisation list (Annex XIV) substances.
It is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.
Supplementary info
- This supplementary information is collated from multiple sources and compiled automatically.
- Activity summary
Frataxin often known by the alternate name FXN is a mitochondrial protein with a mass of approximately 21000 Dalton. It is expressed mainly in tissues with high energy demands like the heart liver and pancreas. Frataxin plays an important role in iron-sulfur cluster assembly which is essential for various cellular processes. The protein is a part of mitochondria where it regulates iron homeostasis and prevents oxidative damage by minimizing iron-induced free radical generation.
- Biological function summary
Several cellular processes depend on the correct function of this protein. Frataxin assists in forming iron-sulfur clusters acting within a multiprotein complex in the mitochondria. The complex includes proteins such as ISCU which are involved in the assembly and repair of iron-sulfur clusters. These clusters are necessary for supporting mitochondrial electron transport and other fundamental metabolic pathways that require iron-sulfur dependencies.
- Pathways
Frataxin's involvement extensively affects the mitochondrial respiratory chain and the mitochondrial biogenesis process. It plays a role in the electron transport chain by stabilizing iron-sulfur-containing complexes. NAB is one associated protein that interacts closely within these pathways sharing a connection through iron-sulfur cluster transportation and assembly systems. Efficient function of these pathways ensures a proper energetic output of cells.
- Associated diseases and disorders
Frataxin mutations are directly linked to Friedreich's ataxia a neurodegenerative disease causing progressive damage to the nervous system. The deficiency or dysfunction in frataxin causes accumulation of iron in mitochondria leading to increased oxidative stress. Another related disorder includes heart disease which emerges due to the same oxidative stress pathway. Proteins such as Nfs1 are also involved sharing the responsibility with frataxin in scavenging excess iron protecting against related tissue damage.
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7 product images
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Example of Frataxin mouse standard curve.
Background-subtracted data values (mean +/- SD) are graphed.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Example of Frataxin mouse standard curve. The Frataxin mouse standard curve was prepared as described. Raw data values are shown in the table. Background-subtracted data values (mean +/- SD) are graphed.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Linearity of dilution of various mouse cell and tissue extracts.
NIH 3T3, RAW264.7 and mouse liver extracts were prepared as described previously and interpolated values corrected by dilution factor (mean +/- SD) are graphed.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Quantitation of mouse Frataxin protein expression in different cell and tissue extract.
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.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Native mouse Frataxin protein was measured in the following biological samples in a 2-fold dilution series. Sample dilutions are made in 1X Cell Extraction Buffer PTR.
Linearity of dilution is determined based on interpolated values from the standard curve. Linearity of dilution defines a sample concentration interval in which interpolated target concentrations are directly proportional to sample dilution.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Interpolated concentration of native Frataxin was measured in duplicate at different sample concentrations in 96-well vs. 384-well plates. Undiluted samples are 10 µg/mL RAW264.7 cell extract. The interpolated dilution factor corrected values are plotted (mean +/- SD, n=2). Sample dilutions are made in 1X Cell Extraction Buffer PTR.
Sandwich ELISA - Mouse Frataxin ELISA Kit (ab199078)
Example of mouse Frataxin standard curve in 96-well vs. 384-well plate. Background-subtracted data values (mean +/- SD) are graphed.
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