AB199078

Mouse Frataxin ELISA Kit

SimpleStep
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(3 Publications)

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

View Alternative Names

Frda, Fxn

9 Images

ELISA - Mouse Frataxin ELISA Kit (AB199078)

ELISA

Supplier Data

ELISA - Mouse Frataxin ELISA Kit (AB199078)

sELISA

Lab

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.

sELISA

Supplier Data

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.

sELISA

Supplier Data

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.

sELISA

Supplier Data

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Representative image of Mouse Frataxin ELISA Kit (ab199078)

Components shown from left to right :

- 10X Wash Buffer PT (ab206977)

- 10X Mouse Frataxin Capture Antibody

- 10X Mouse Frataxin Detector Antibody

- Mouse Frataxin Lyophilised Purified Protein (2 vials)

- 50X Cell Extraction Enhancer Solution (ab193971)

- Sample diluent NS

- TMB solution

- Stop Solution

- 5X Cell Extraction Buffer PTR (ab193970)

- Antibody diluent 5BI

SimpleStep Pre-Coated 96-Well Microplate (ab206978) - shown behind bottles / vials.

Plate Seals - shown underneath bottles / vials.

Note : The vial labels shown in this image use generic names for illustrative purposes only and may not exactly match the specific component names included in the ELISA kit.

Note : Colors of solutions in image may not precisely match the shade of colors in the actual kit

sELISA

Lab

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.

sELISA

Supplier Data

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.

sELISA

Supplier Data

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Example of Frataxin mouse standard curve.

Background-subtracted data values (mean +/- SD) are graphed.

sELISA

Supplier Data

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.

Key facts

Detection method

Colorimetric

Sample types

Cell culture extracts, Tissue Extracts

Reacts with

Mouse

Assay type

Sandwich (quantitative)

Sensitivity

= 5 pg/mL

Range

31.25 - 2000 pg/mL

Assay time

1h 30m

Assay Platform

Microplate (12 x 8 well strips)

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "sELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }

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Product details

Mouse Frataxin ELISA Kit ab199078 is a rapid single-wash 90-min Sandwich ELISA to measure Mouse Frataxin in cell culture extracts, tissue extracts. This SimpleStep sensitivity is 5 pg/mL.

How the assay works

Mouse 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

Our SimpleStep ELISA^® kits use recombinant monoclonal antibodies rigorously validated to ensure the highest level of consistency and reproducibility, improved sensitivity and specificity and ease of scalability and security of supply.
Please refer to our protocol booklet for more details.

Mouse Frataxin ELISA Kit ab199078 protocol summary

1. Mix: add samples/standards to the wells together with the capture and detector antibody cocktail. Incubate 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 450 nm

Save your precious samples

Our 384-well Mouse Frataxin ELISA Kit only requires a maximum of 12.5µL sample

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.

REACH authorisation
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.

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Precision

[ { "reproducibilityType": "Inter", "sample": "NIH 3T3", "replicates": 3, "mean": null, "standardDeviation": null, "coefficientOfVariability": "2" }, { "reproducibilityType": "Intra", "sample": "NIH 3T3", "replicates": 3, "mean": null, "standardDeviation": null, "coefficientOfVariability": "1.3" } ]

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Recovery

[ { "sample": "Fetal Bovine Serum", "range": "99 - 103 %", "average": "= 101" }, { "sample": "Serum", "range": "95 - 109 %", "average": "= 100" }, { "sample": "serum free media", "range": "99 - 103 %", "average": "= 101" } ]

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What's included?

{ "values": { "1x384Tests": { "sellingSize": "1 x 384 Tests", "publicAssetCode":"ab199078-1x384Tests", "assetComponentDetails": [ { "size":"2 x 12 mL", "name":"Stop Solution", "number":"AB199078-CMP04" }, { "size":"1 x 600 µL", "name":"10X Mouse Frataxin Capture Antibody", "number":"AB199078-CMP09" }, { "size":"2 x 1 Vial", "name":"Mouse Frataxin Lyophilized Recombinant Protein", "number":"AB199078-CMP08" }, { "size":"1 x 20 mL", "name":"10X Wash Buffer PT (ab206977)", "number":"AB199078-CMP12" }, { "size":"1 x 6 mL", "name":"Antibody Diluent 5BI", "number":"AB199078-CMP02" }, { "size":"1 x 1 Unit", "name":"384 well CaptSure™ microplates", "number":"AB199078-CMP14" }, { "size":"6 x 1 mL", "name":"50X Cell Extraction Enhancer Solution (ab193971)", "number":"AB199078-CMP06" }, { "size":"1 x 50 mL", "name":"5X Cell Extraction Buffer PTR (ab193970)", "number":"AB199078-CMP07" }, { "size":"1 x 1 Unit", "name":"Plate Seals", "number":"AB199078-CMP11" }, { "size":"1 x 600 µL", "name":"10X Mouse Frataxin Detector Antibody", "number":"AB199078-CMP10" }, { "size":"2 x 12 mL", "name":"TMB Development Solution", "number":"AB199078-CMP05" } ] }, "10x96Tests": { "sellingSize": "10 x 96 Tests", "publicAssetCode":"ab199078-10x96Tests", "assetComponentDetails": [ { "size":"1 x 120 mL", "name":"Stop Solution", "number":"AB199078-CMP04" }, { "size":"10 x 600 µL", "name":"10X Mouse Frataxin Capture Antibody", "number":"AB199078-CMP09" }, { "size":"1 x 200 mL", "name":"10X Wash Buffer PT (ab206977)", "number":"AB199078-CMP12" }, { "size":"10 x 2 Vial", "name":"Mouse Frataxin Lyophilized Recombinant Protein", "number":"AB199078-CMP08" }, { "size":"10 x 6 mL", "name":"Antibody Diluent 5BI", "number":"AB199078-CMP02" }, { "size":"2 x 50 mL", "name":"5X Cell Extraction Buffer PTR (ab193970)", "number":"AB199078-CMP07" }, { "size":"10 x 1 mL", "name":"50X Cell Extraction Enhancer Solution (ab193971)", "number":"AB199078-CMP06" }, { "size":"10 x 600 µL", "name":"10X Mouse Frataxin Detector Antibody", "number":"AB199078-CMP10" }, { "size":"1 x 12 mL", "name":"TMB Development Solution", "number":"AB199078-CMP05" }, { "size":"10 x 1 Unit", "name":"SimpleStep Pre-Coated 96-Well Microplate (ab206978)", "number":"AB199078-CMP03" }, { "size":"10 x 1 Unit", "name":"Plate Seals", "number":"AB199078-CMP11" } ] }, "1x96Tests": { "sellingSize": "1 x 96 Tests", "publicAssetCode":"ab199078-1x96Tests", "assetComponentDetails": [ { "size":"1 x 12 mL", "name":"Stop Solution", "number":"AB199078-CMP04", "productcode":"" }, { "size":"1 x 20 mL", "name":"10X Wash Buffer PT (ab206977)", "number":"AB199078-CMP12", "productcode":"" }, { "size":"1 x 1 mL", "name":"50X Cell Extraction Enhancer Solution (ab193971)", "number":"AB199078-CMP06", "productcode":"" }, { "size":"1 x 12 mL", "name":"Sample Diluent NS (ab193972)", "number":"AB199078-CMP13", "productcode":"" }, { "size":"1 x 12 mL", "name":"TMB Development Solution", "number":"AB199078-CMP05", "productcode":"" }, { "size":"1 x 1 Unit", "name":"Plate Seals", "number":"AB199078-CMP11", "productcode":"" }, { "size":"1 x 600 µL", "name":"10X Mouse Frataxin Capture Antibody", "number":"AB199078-CMP09", "productcode":"" }, { "size":"1 x 6 mL", "name":"Antibody Diluent 5BI", "number":"AB199078-CMP02", "productcode":"" }, { "size":"2 x 1 Vial", "name":"Mouse Frataxin Lyophilized Recombinant Protein", "number":"AB199078-CMP08", "productcode":"" }, { "size":"1 x 1 Unit", "name":"SimpleStep Pre-Coated 96-Well Microplate (ab206978)", "number":"AB199078-CMP03", "productcode":"" }, { "size":"1 x 10 mL", "name":"5X Cell Extraction Buffer PTR (ab193970)", "number":"AB199078-CMP07", "productcode":"" }, { "size":"1 x 600 µL", "name":"10X Mouse Frataxin Detector Antibody", "number":"AB199078-CMP10", "productcode":"" } ] } } }

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Properties and storage information

Shipped at conditions

Blue Ice

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

+4°C

Storage information

+4°C

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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

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.

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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Product protocols

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Target data

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.

See full target information Fxn

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Publications (3)

Recent publications for all applications. Explore the full list and refine your search

Disease models & mechanisms 16: PubMed37691621

2023

Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.

Applications

Unspecified application

Species

Unspecified reactive species

Nicole M Sayles,Jill S Napierala,Josef Anrather,Nadège Diedhiou,Jixue Li,Marek Napierala,Hélène Puccio,Giovanni Manfredi

Molecular therapy. Methods & clinical development 19:120-138 PubMed33209958

2020

High Levels of Frataxin Overexpression Lead to Mitochondrial and Cardiac Toxicity in Mouse Models.

Applications

Unspecified application

Species

Unspecified reactive species

Brahim Belbellaa,Laurence Reutenauer,Nadia Messaddeq,Laurent Monassier,Hélène Puccio

Molecular therapy : the journal of the American So 26:1940-1952 PubMed29853274

2018

Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia.

Applications

Unspecified application

Species

Unspecified reactive species

Françoise Piguet,Charline de Montigny,Nadège Vaucamps,Laurence Reutenauer,Aurélie Eisenmann,Hélène Puccio

View all publications

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websiteProtocolBooklet

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Product promise

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Mouse Frataxin ELISA Kit

ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Sandwich ELISA - Mouse Frataxin ELISA Kit (AB199078)

Key facts

Detection method

Sample types

Reacts with

Assay type

Sensitivity

Range

Assay time

Assay Platform

Reactivity data

Product details

Precision

Recovery

What's included?

Properties and storage information

Shipped at conditions

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (3)

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