{"product_id":"abcam-ab212062","title":"Abcam, ab212062, Human Frataxin Matched Antibody Pair Kit","description":"\u003cp\u003eSize: 5 x 96Tests\u003cbr\u003e\nHuman Frataxin Matched Antibody Pair Kit is a kit for ELISA development for the measurement of Human Frataxin by sandwich ELISA method.\u003cbr\u003e\nKey facts\u003cbr\u003e\nDetection method:Colorimetric,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nAssay type:ELISA set,\u003cbr\u003e\nSensitivity:= 8.4 pg\/mL,\u003cbr\u003e\nRange:39 - 2500 pg\/mL,\u003cbr\u003e\nAssay Platform:Reagents\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nHuman 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 . An accessory pack can be purchased which includes buffer reagents required to perform 10 x 96-well plate sandwich ELISAs (\u003cbr\u003e\nab210905\u003cbr\u003e\n).For additional information on the performance of the antibody pair used in this kit, please see our equivalent SimpleStep ELISA kit\u003cbr\u003e\nab176112\u003cbr\u003e\n. 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.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Blue Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C, Storage information--20°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nFrataxin 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.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nSeveral 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.\u003cbr\u003e\nPathways\u003cbr\u003e\nFrataxin'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.\u003cbr\u003e\nFrataxin 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.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46855626064041,"sku":"ab212062","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab212062","provider":"Iright","version":"1.0","type":"link"}