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AB229412

Human Frataxin ELISA Kit, Fluorescent

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Human Frataxin ELISA Kit, Fluorescent is a single-wash 90-min Simplestep used to quantify Human Frataxin with a sensitivity of 1.7 pg/ml. The assay uses a simple mix-wash-read protocol with just one incubation and one wash step.

- Fluorescent Sandwich ELISA - 530/570/590 nm readout : works on any standard plate reader

View Alternative Names

FRDA, X25, FXN, Friedreich ataxia protein, Fxn

4 Images
Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)
  • sELISA

Unknown

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)

Quantification of Frataxin expression in different cell lines.

Interpolated values of Frataxin are plotted for the indicated cell lines based on an extract load of 10 μg/mL.

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)
  • sELISA

Unknown

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)

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

Background subtracted data from duplicate measurements are plotted.

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)
  • sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)

Example of human Frataxin standard curve

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)
  • sELISA

Unknown

Sandwich ELISA - Human Frataxin ELISA Kit, Fluorescent (AB229412)

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.

Key facts

Detection method

Fluorescent

Sample types

Cell culture extracts, Tissue Extracts

Reacts with

Human

Assay type

Sandwich

Results type

Quantitative

Sensitivity

= 1.7 pg/mL

Range

3.91 - 4000 pg/mL

Assay time

1h 30m

Assay Platform

Pre-coated microplate (12 x 8 well strips)

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Primary Antibodies

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Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Frataxin antibody [EPR21840] (AB219414)

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Secondary Antibodies

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Goat Anti-Mouse IgG H&L (Alexa Fluor® 488)

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Primary Antibodies

AB9485

Anti-GAPDH antibody - Loading Control

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  • 5
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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

Frataxin in vitro CatchPoint SimpleStep ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of Frataxin protein in human cell and tissue extracts.

This CatchPoint SimpleStep ELISA kit has been optimized for Molecular Devices Microplate Readers. Click here for a list of recommended Microplate Readers.
If using a Molecular Devices' plate reader supported by SoftMax® Pro software, a preconfigured protocol for these CatchPoint SimpleStep ELISA Kits is available with all the protocol and analysis settings at www.softmaxpro.org.

The CatchPoint SimpleStep ELISA employs an affinity tag labeled capture antibody and a reporter conjugated detector antibody which immunocapture the sample analyte in solution. This entire complex (capture antibody/analyte/detector antibody) is in turn immobilized via immunoaffinity of an anti-tag antibody coating the well. To perform the assay, samples or standards are added to the wells, followed by the antibody mix. After incubation, the wells are washed to remove unbound material. CatchPoint HRP Development Solution containing the Stoplight Red Substrate is added. During incubation, the substrate is catalyzed by HRP generating a fluorescent product. Signal is generated proportionally to the amount of bound analyte and the intensity is measured in a fluorescence plater reader at 530/570/590 nm Excitation/Cutoff/Emission.

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

[ { "sample": "EDTA Plasma", "range": "88 - 92 %", "average": "= 89" }, { "sample": "Serum", "range": "86 - 98 %", "average": "= 92" } ]
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What's included?

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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 : 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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