{"product_id":"abcam-ab201121","title":"Abcam, ab201121, Human Frataxin ELISA Kit (10 x 96 well plate)","description":"\u003cp\u003eSize: 10 x 96Tests\u003cbr\u003e\nHuman Frataxin ELISA Kit (10 x 96 well plate) is a single-wash 90-min Simplestep used to quantify Human Frataxin (10 x 96 well plate) with a sensitivity of 2.1 pg\/ml. The assay uses a simple mix-wash-read protocol with just one incubation and one wash step. - Colorimetric Sandwich ELISA - 450 nm readout : works on any standard plate reader - Different formats for different needs: 10x96 plates for bulk orders\u003cbr\u003e\nKey facts\u003cbr\u003e\nDetection method:Colorimetric,\u003cbr\u003e\nSample types:Cell culture extracts, Tissue Extracts,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nAssay type:Sandwich (quantitative),\u003cbr\u003e\nSensitivity:= 2.1 pg\/mL,\u003cbr\u003e\nRange:9.88 - 800 pg\/mL,\u003cbr\u003e\nAssay time:1h 30m,\u003cbr\u003e\nAssay Platform:Microplate\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nHuman Frataxin ELISA Kit (10 x 96 well plate) ab201121 is a rapid single-wash 90-min Sandwich ELISA to measure Human Frataxin (10 x 96 well plate)in cell culture extracts, tissue extracts. This SimpleStep sensitivity is 2.1 pg\/mL.\u003cbr\u003e\nHow the assay works\u003cbr\u003e\nHuman Frataxin (10 x 96 well plate)SimpleStep ELISA\u003cbr\u003e\nemploys capture antibodies conjugated to an affinity tag that is recognized by the monoclonal antibody used to coat our SimpleStep ELISA\u003cbr\u003e\nplates. This approach to sandwich ELISA allows the formation of the antibody-analyte sandwich complex in a single step, significantly reducing assay time. See the SimpleStep ELISA\u003cbr\u003e\nprotocol summary in the image section for further details.\u003cbr\u003e\nAssay Specificity\u003cbr\u003e\nOur SimpleStep ELISA\u003cbr\u003e\nkits use recombinant monoclonal antibodies rigorously validated to ensure the highest level of consistency and reproducibility, improved sensitivity and specificity and ease of scalability and security of supply.\u003cbr\u003e\nPlease refer to our protocol booklet for more details.\u003cbr\u003e\nHuman Frataxin ELISA Kit (10 x 96 well plate) ab201121 protocol summary\u003cbr\u003e\n1. Mix: add samples\/standards to the wells together with the capture and detector antibody cocktail. Incubate 1 hr at room temperature\u003cbr\u003e\n2. Wash\u003cbr\u003e\n3. Add TMB development solution - incubate for 10 min\u003cbr\u003e\n4. Add Stop solution\u003cbr\u003e\n5. Read the results on a plate reader at 450 nm\u003cbr\u003e\nFrataxin is a 17 kDa nuclear-encoded mitochondrial protein. In humans 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 human 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.\u003cbr\u003e\nREACH authorisation\u003cbr\u003e\nAbcam has not and does not intend to apply for the REACH Authorisation of customers' uses of products that contain European Authorisation list (Annex XIV) substances.\u003cbr\u003e\nIt is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Dry Ice, Appropriate short-term storage conditions-Multi, Appropriate long-term storage conditions-Multi, Storage information-Please refer to protocols\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":46855636811945,"sku":"ab201121","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab201121","provider":"Iright","version":"1.0","type":"link"}