OK to freeze
This product has been discontinued.
Frataxin (Q16595) is encoded by the gene FXN on chromosome 9. Expression has been shown in many tissues where the protein is imported into the mitochondrial matrix and processed to a mature 17.3 kDa protein. The role of frataxin is not clear, however, it has been proposed to function as an iron chaperone, an iron storage protein, as a repair enzyme of damaged FeS clusters in aconitase, as a defense against oxidative stress and activator of mitochondrial oxidative phosphorylation.
Partial frataxin deficiency in humans results in Friedrich ataxia (FRDA), an autosomal recessive disease characterized mainly by progressive neurodegeneration in the spinal cord, and frequently by hypertrophic cardiomyopathy and diabetes. FRDA is the most common form of ataxia (1:30,000 1:50,000 in US and Europe). In greater than 95% of cases the genetic basis of the deficiency is a triplet-nucleotide repeat of GAA in intron 1 of both frataxin alleles. Normal individuals carry ′small number repeats′ (6-12) while less than 20% of normal individuals contain ′large normal repeats′ (14-34). The role of these repeats, if any, is unknown. Individuals with large number repeats are at increased risk of having offspring with expanded repeats (66-1500) which result in disease. Expanded repeats interfere with transcription of frataxin mRNA and result in a decrease in frataxin protein causing at the cellular level impairment of energy metabolism, increased oxidative stress, reduced heme biosynthesis, impaired iron metabolism and iron accumulation in the heart and nervous system at the tissue level.
In this assay, Frataxin is immunocaptured within the wells. The quantity of protein is measured by adding a second frataxin specific antibody that is labeled with horseradish peroxidase. This peroxidase changes the substrate from colorless to blue. The rate of color development is proportional to the amount of protein captured in the well and can be monitored at 600 nm. Alternatively, the assay can be terminated, at a user defined time, by the addition of 1N HCl (not supplied) and the assay performed as an end point measurement at 450 nm.
• 20 ml 20X Buffer, (72602-20ML)• 8 ml 10X Blocking Solution, (72603-8ML)• 20 ml HRP Development Solution, (72604-20ML)• 1 ml Detergent, (72605-1ML)• 1 ml 20X Detector Antibody, (72606-1ML)• 1 ml 20X HRP Label, (72607-1ML)• 1 ea Microplate, (72608-1EA)
The WideScreen Frataxin ELISA Kit contains sufficient materials for 96 measurements (one 96-well microplate). This kit contains the necessary buffers and reagents for the respective reaction. The kit also contains a 96-well microplate with a monoclonal antibody pre-bound to the wells of the microplate. This plate can be broken into 12 separate 8-well strips for convenience; therefore the plate can be used for up to 12 separate experiments.
Storage: Store all components at 4°C. The kit is stable for at least 6 months.