Human Frataxin Matched Antibody Pair Kit (ab212062)
Key features and details
- Unlabeled capture antibody, biotin-labeled detection antibody and calibrated protein standard
- For economical ELISA and ELISA-based assay development
- Reacts with: Human
- Range: 39 pg/ml - 2500 pg/ml
Overview
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Product name
Human Frataxin Matched Antibody Pair Kit
See all Frataxin kits -
Detection method
Colorimetric -
Assay type
ELISA set -
Sensitivity
8.4 pg/ml -
Range
39 pg/ml - 2500 pg/ml -
Species reactivity
Reacts with: Human -
Product overview
Human Frataxin Matched Antibody Pair Kits include a capture and a biotinylated detector antibody pair, along with a calibrated protein standard, suitable for sandwich ELISA. The Matched Antibody Pair Kit can be used to quantify native and recombinant human Frataxin.
Optimization of the kit reagents to sample type, immunoassay format or instrumentation may be required. Guidelines for use of this kit in a standard 96-well microplate sandwich ELISA using HRP/TMB system of colorimetric detection is described in this assay procedure for the purposes of quantification.
Protocol information and tips on the use of the Matched Antibody Pair kits for sandwich ELISA can be found on our website. An accessory pack can be purchased which includes buffer reagents required to perform 10 x 96-well plate sandwich ELISAs (ab210905).
For additional information on the performance of the antibody pair used in this kit, please see our equivalent SimpleStep ELISA kit ab176112. Please note that while the antibody pair is the same provided in the corresponding SimpleStep ELISA Kit, due to differences in their formulation, this antibody pair cannot be used with the consumables provided with our SimpleStep ELISA Kits.
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Tested applications
Suitable for: ELISA, IAmore details -
Platform
Reagents
Properties
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Storage instructions
Store at -20°C. Please refer to protocols. -
Components 10 x 96 tests 5 x 96 tests Human Frataxin Capture Antibody 2 x 50µg 1 x 50µg Human Frataxin Detector Antibody 2 x 12.5µg 1 x 12.5µg Human Frataxin Lyophilized Protein 2 vials 1 vial -
Research areas
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Function
Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. 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. Modulates the RNA-binding activity of ACO1. -
Tissue specificity
Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. -
Involvement in disease
Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. -
Sequence similarities
Belongs to the frataxin family. -
Post-translational
modificationsProcessed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. -
Cellular localization
Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. - Information by UniProt
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Alternative names
- CyaY
- d-FXN
- FA
see all -
Database links
- Entrez Gene: 2395 Human
- Omim: 606829 Human
- SwissProt: Q16595 Human
- Unigene: 20685 Human
Images
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Human Frataxin standard curve.
Standard calibration curve. Background subtracted values are graphed
