AB201121

Human Frataxin ELISA Kit (10 x 96 well plate)

SimpleStep
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Human Frataxin ELISA Kit (10 x 96 well plate) is a single-wash 90-min Simplestep used to quantify Human Frataxin (10 x 96 well plate) 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: 10x96 plates for bulk orders

View Alternative Names

FRDA, X25, FXN, Friedreich ataxia protein, Fxn

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ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

ELISA

Supplier Data

ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

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.

sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Titration of HeLa cell extract within the working range of the assay.

Background subtracted data from duplicate measurements are plotted.

sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

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.

sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Example of Frataxin standard curve.

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

Key facts

Detection method

Colorimetric

Sample types

Cell culture extracts, Tissue Extracts

Reacts with

Human

Assay type

Sandwich

Results type

Quantitative

Sensitivity

= 2.1 pg/mL

Range

9.88 - 800 pg/mL

Assay time

1h 30m

Assay Platform

Microplate

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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

Human Frataxin ELISA Kit (10 x 96 well plate) ab201121 is a rapid single-wash 90-min Sandwich ELISA to measure Human Frataxin (10 x 96 well plate)in cell culture extracts, tissue extracts. This SimpleStep sensitivity is 2.1 pg/mL.

How the assay works

Human Frataxin (10 x 96 well plate)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.

Human Frataxin ELISA Kit (10 x 96 well plate) ab201121 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

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.

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": "HeLa", "replicates": 3, "mean": null, "standardDeviation": null, "coefficientOfVariability": "6.3" }, { "reproducibilityType": "Intra", "sample": "HeLa", "replicates": 9, "mean": null, "standardDeviation": null, "coefficientOfVariability": "2.3" } ]

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Recovery

[ { "sample": "Cell culture media", "range": "93 - 101 %", "average": "= 97" }, { "sample": "EDTA Plasma", "range": "88 - 92 %", "average": "= 89" }, { "sample": "Serum", "range": "86 - 98 %", "average": "= 92" } ]

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What's included?

{ "values": { "10x96Tests": { "sellingSize": "10 x 96 Tests", "publicAssetCode":"ab201121-10x96Tests", "assetComponentDetails": [ { "size":"10 x 1 Unit", "name":"SimpleStep Pre-Coated 96-Well Microplate (ab206978)", "number":"AB201121-CMP05", "productcode":"" }, { "size":"1 x 5 mL", "name":"50X Cell Extraction Enhancer Solution (ab193971)", "number":"AB201121-CMP13", "productcode":"" }, { "size":"1 x 120 mL", "name":"Sample Diluent NS (ab193972)", "number":"AB201121-CMP16", "productcode":"" }, { "size":"10 x 1 Unit", "name":"Plate Seals", "number":"AB201121-CMP10", "productcode":"" }, { "size":"1 x 60 mL", "name":"Antibody Diluent 4BI", "number":"AB201121-CMP15", "productcode":"" }, { "size":"1 x 6 mL", "name":"10X Human Frataxin Capture Antibody", "number":"AB201121-CMP01", "productcode":"" }, { "size":"1 x 200 mL", "name":"10X Wash Buffer PT (ab206977)", "number":"AB201121-CMP11", "productcode":"" }, { "size":"1 x 2 mL", "name":"Human Frataxin Recombinant Protein", "number":"AB201121-CMP12", "productcode":"" }, { "size":"1 x 50 mL", "name":"5X Cell Extraction Buffer PTR (ab193970)", "number":"AB201121-CMP14", "productcode":"" }, { "size":"1 x 6 mL", "name":"10X Human Frataxin Detector Antibody", "number":"AB201121-CMP02", "productcode":"" }, { "size":"1 x 120 mL", "name":"TMB Development Solution", "number":"AB201121-CMP07", "productcode":"" }, { "size":"1 x 120 mL", "name":"Stop Solution", "number":"AB201121-CMP06", "productcode":"" } ] } } }

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Properties and storage information

Shipped at conditions

Dry Ice

Appropriate short-term storage conditions

Multi

Appropriate long-term storage conditions

Multi

Storage information

Please refer to protocols

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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 : 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).

See full target information FXN

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Product promise

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Human Frataxin ELISA Kit (10 x 96 well plate)

ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Sandwich ELISA - Human Frataxin ELISA Kit (10 x 96 well plate) (AB201121)

Key facts

Detection method

Sample types

Reacts with

Assay type

Results type

Sensitivity

Range

Assay time

Assay Platform

Complementary Products

Assay Kits

Primary Antibodies

Secondary Antibodies

Primary Antibodies

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

Product promise