Abcam, ab229424, Mouse Frataxin ELISA Kit, Fluorescent

Size: 1 x 96Tests
Mouse Frataxin ELISA Kit, Fluorescent is a single-wash 90-min Simplestep used to quantify Mouse Frataxin with a sensitivity of 4.8 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
Key facts
Detection method:Fluorescent,
Sample types:Cell culture extracts, Tissue Extracts,
Reacts with:Mouse,
Assay type:Sandwich (quantitative),
Sensitivity:= 4.8 pg/mL,
Range:7.8 - 16000 pg/mL,
Assay time:1h 30m,
Assay Platform:Pre-coated microplate (12 x 8 well strips)

Product details:
Frataxin
in vitro
CatchPoint SimpleStep ELISA (Enzyme-Linked Immunosorbent Assay) kit is designed for the quantitative measurement of Frataxin protein in mouse cell and tissue extracts.
This CatchPoint SimpleStep ELISA kit has been
optimized for Molecular Devices Microplate Readers
. Click
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.
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
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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.

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

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.