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Download our Frataxin Brochure. Solutions for monitoring Friedreich's ataxia patients or cell models, inlcuding measurement of frataxin levels and downstream effects of frataxin dysregulation.



Frataxin


UniProt Number: Q16595
Alternate Names: Friedreich ataxia protein, FXN, FRDA, X25, Cleaved into the following 3 chains: Frataxin intermediate form, Frataxin(56-210), Frataxin(81-210)
Structure and Function: Frataxin 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).
Disease Associations: Defects in FXN are the cause of Friedreich ataxia (FA) [MIM:229300]. FA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia.


Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MSF01 Anti-Frataxin antibody [18A5DB1] human WB, ICC, FLOW 100 µg
MSF02 Anti-Frataxin antibody [17A11] human, mouse, rat, cow, dog WB, ICC, FLOW 100 µg
ab156033 Anti-Frataxin antibody [18A5DB1] (Alexa Fluor® 488) human ICC, FLOW 100 µg


Protein Quantity Assays
Cat. No. Name Reactivity Amount
MS956 Frataxin multispecies ELISA Kit human, mouse, rat, cow, dog 96 tests
MSF41 Frataxin human ELISA Kit human 96 tests
MSF31 Frataxin Protein Quantity Dipstick Assay Kit human 30 tests
90 tests


Multiplexing Arrays
Cat. No. Name Reactivity Amount
MSX31 MetaPath™ MitoDisease
4-Plex Dipstick Array
human 30 tests
90 tests
MSX32 MetaPath™ Fatty Acid Oxdiation 4-Plex Dipstick Array human 30 tests
90 tests


Recombinant Proteins
Cat. No. Name Source Amount
MSF42 Frataxin Recombinant Protein human 12 ng



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