SDS-PAGE Analysis Purified Frataxin Recombinant Mouse Monoclonal Antibody (rFXN/2124). Confirmation of Purity and Integrity of Antibody.
Known Applications & Suggested Dilutions
Host / Ig Isotype
Mol. Weight of Antigen
Specificity & Comments
Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich's ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix suggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet.