An antibody raised against frataxin; the peptide promotes the biosynthesis of heme plus assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways.
Catalogue number crb2005652 Antibody Anti-Frataxin antibody Antigen Peptide KLH conjugated synthetic peptide crb1200861e Protein ID Q16595 Human FXN, O35943 Mouse Fxn Aliases Frataxin (mitochondrial), FXN, Fxn, Frda, Frataxin intermediate form, Frataxin mature form Cross-Reactivity Human Host Species Rabbit Antibody Type Polyclonal Concentration 1mg/ml Glycine (R1G), 1mg/ml Glycine (R2G) Target Frataxin
Frataxin is a vital iron chaperone and regulator of iron-dependent pathways. Within mitochondria, frataxin protects aconitase iron sulfer clusters ([4Fe-4S]2+, also known as ISCs) and aids the enzymatic reactivation for heme biosynthesis. The mitochondria require a consistent and regulated supply of iron to the ISC for heme production and prevention of oxidative stress catalysed by free iron. Frataxin interacts with many of the ISC biogenesis proteins such as Isu1, GRP75, ferrochelatase, and aconitase itself. Outside the mitochondria, a pool of frataxin has been identified that suppresses the apoptotic response.
Mutations of frataxin have been characterised. Depleted levels lead to defective ISCs, heme biosynthesis, unregulated iron stores in the mitochondria causing oxidative damage and stress induced apoptosis. The hereditary disorder Friedreich’s Ataxia (FRDA) is caused by a lack of sufficient frataxin production. This is leads to cardiomyopathy and often diabetes.