Frataxin Protein Quantity Dipstick Assay Kit is an immunoassay kit for the detection of Frataxin Protein Quantity in Human in Whole Blood samples.
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- 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
- Scientific reports 8:172172018FAST-1 antisense RNA epigenetically alters FXN expression.Applications:Unspecified applicationReactive species:Unspecified reactive speciesHajar Mikaeili et. al.PubMed 30464193
- Molecular therapy. Nucleic acids 12:19-322018Increased Frataxin Expression Induced in Friedreich Ataxia Cells by Platinum TALE-VP64s or Platinum TALE-SunTag.Applications:Unspecified applicationReactive species:Unspecified reactive speciesKhadija Cherif et. al.PubMed 30195758
- Aging 9:1440-14522017Friedreich's ataxia induced pluripotent stem cell-derived cardiomyocytes display electrophysiological abnormalities and calcium handling deficiency.Applications:Unspecified applicationReactive species:Unspecified reactive speciesDuncan E Crombie,Claire L Curl,Antonia Ja Raaijmakers,Priyadharshini Sivakumaran,Tejal Kulkarni,Raymond Cb Wong,Itsunari Minami,Marguerite V Evans-Galea,Shiang Y Lim,Lea Delbridge,Louise A Corben,Mirella Dottori,Norio Nakatsuji,Ian A Trounce,Alex W Hewitt,Martin B Delatycki,Martin F Pera,Alice PébayPubMed 28562313
- Gene therapy 23:606-142016Development of an AAV9 coding for a 3XFLAG-TALEfrat#8-VP64 able to increase in vivo the human frataxin in YG8R mice.
- Orphanet journal of rare diseases 10:1082015Friedreich ataxia in Norway - an epidemiological, molecular and clinical study.Applications:Unspecified applicationReactive species:HumanIselin Marie Wedding et. al.PubMed 26338206
- Molecular therapy. Methods & clinical development 1:140442014An AAV9 coding for frataxin clearly improved the symptoms and prolonged the life of Friedreich ataxia mouse models.Applications:Unspecified applicationReactive species:Unspecified reactive speciesCatherine Gérard,Xiao Xiao,Mohammed Filali,Zoé Coulombe,Marie Arsenault,Jacques Couet,Juan Li,Marie-Claude Drolet,Pierre Chapdelaine,Amina Chikh,Jacques P TremblayPubMed 26015982
- Human molecular genetics 23:6848-622014Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.Applications:Unspecified applicationReactive species:Unspecified reactive speciesSunil Sahdeo et. al.PubMed 25113747
- PloS one 9:e1017182014Functional characterization of Friedreich ataxia iPS-derived neuronal progenitors and their integration in the adult brain.Applications:Unspecified applicationReactive species:Unspecified reactive speciesMatthew J Bird et. al.PubMed 25000412
- PloS one 9:e894882014Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models.Applications:Unspecified applicationReactive species:Unspecified reactive speciesChiranjeevi Sandi et. al.PubMed 24586819
Key facts
- Sample types
- Whole Blood
- Assay type
- Sandwich (quantitative)
- Reactive species
- Human
Reactivity data
| Application | Reactivity | Dilution info | Notes |
|---|---|---|---|
Application sELISA | Reactivity Reacts | Dilution info - | Notes - |
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
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Frataxin Protein Quantity Dipstick Assay Kit is an immunoassay kit for the detection of Frataxin Protein Quantity in Human in Whole Blood samples.
Key facts
- Sample types
- Whole Blood
- Assay type
- Sandwich (quantitative)
- Reactive species
- Human
- Assay Platform
- Reagents
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
ab109881 is used to rapidly quantify frataxin protein levels from human sample materials. Purification of mitochondria is not necessary for the performance of this assay. Based on the immunologic sandwich assay, the kit utilizes two monoclonal antibodies (mAbs) specific to different antigens present on the mature form of frataxin. One antibody is immobilized on the nitrocellulose membrane of the dipstick in a thin line perpendicular to the length of the dipstick while the other is gold-conjugated which gives a visual signal. When frataxin is present in the sample, a red-colored line appears on the dipstick at the site of the anti-frataxin mAb immobilized on the membrane. The signal intensity is directly related to the level of frataxin in the sample. The signal intensity is best measured by a dipstick reader or may be analyzed by another imaging system.
All components are stable in their provided containers at room temperature out of direct sunlight.
After diluting the 10X Blocking Buffer to 2X, store at 4°C.
For long-term storage, all buffers can be stored at 4°C.
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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Sandwich ELISA - Frataxin Protein Quantity Dipstick Assay Kit (ab109881)
An example using ab109881 to quantify frataxin levels from ß -lymphocyte cell culture samples derived from control individuals and Friedreich's Ataxia (FA) patients.
Based on the above standard curve values, of protein extract for the controls and FA patient samples. (Note: for a statistical analysis, it is preferred to use two dipsticks for each sample; intra-assay CV's are typically <=10%.) For this analysis, the FA patients had between 550 - 925 GAA repeats on the smaller allele. Using GraphPad software, the signal intensity from the standard curve was interpolated and the relative amount of frataxin in the patients, as compared to the controls, was determined. Based on the above analysis, the patient samples had between 13% and 20% of frataxin levels compared to the control.
Sandwich ELISA - Frataxin Protein Quantity Dipstick Assay Kit (ab109881)
An example using ab109881 to quantify frataxin levels from ß -lymphocyte cell culture samples derived from control individuals and Friedreich's Ataxia (FA) patients.
Shown is a 1:2 dilution series using a positive control sample. Approximately 6 to 8 dipsticks are suitable for covering the entire working range and the blank for background levels. In this example the dilution series starts with 4 µg of control sample. A one-site hyperbola line was generated for best-fit analysis using GraphPad.
Sandwich ELISA - Frataxin Protein Quantity Dipstick Assay Kit (ab109881)
Dipstick assays use the well-established lateral flow concept, whereby capture antibodies are striped onto nitrocellulose membrane and a Whatman paper wicking pad draws the sample through the antibody bands. Detector antibodies, conjugated to gold, are dried in the wells of a 96-well plate. Sample is added to the well, the dipstick inserted, and within minutes the line for each target is revealed as the protein-detector antibody-gold complex binds with the capture antibodies. Multiplexing dipstick assays have multiple target protein lines. A positive control goat anti-mouse antibody line is included on all assays to ensure that adequate wicking of the sample occurred.
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