{"product_id":"abcam-ab195175","title":"Abcam, ab195175, Recombinant Mouse Frataxin protein","description":"\u003cp\u003eSize: 20µg\u003cbr\u003e\nRecombinant Mouse Frataxin protein is a Mouse Fragment protein, in the 78 to 207 aa range, expressed in Escherichia coli, with \u0026gt;99%, suitable for SDS-PAGE, sELISA.\u003cbr\u003e\nKey facts\u003cbr\u003e\nPurity:\u0026gt;99% SDS-PAGE,\u003cbr\u003e\nExpression system:Escherichia coli,\u003cbr\u003e\nTags:His tag N-Terminus,\u003cbr\u003e\nApplications:SDS-PAGE, sELISASee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nBiologically active:No,\u003cbr\u003e\nAccession:O35943,\u003cbr\u003e\nAnimal free:No,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nSpecies:Mouse,\u003cbr\u003e\nStorage buffer:pH: 7.5Constituents: 10% Trehalose, 8.06% Sodium chloride, 2.16% Sodium phosphate dibasic heptahydrate, 0.2% Potassium chloride, 0.2% Potassium phosphate monobasic\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nProduct was previously marketed under the MitoSciences sub-brand.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Dry Ice, Appropriate short-term storage conditions--80°C, Appropriate long-term storage conditions--80°C, Storage information-Avoid freeze \/ thaw cycle\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":46843992834217,"sku":"ab195175","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab195175","provider":"Iright","version":"1.0","type":"link"}