Anti-Frataxin antibody

  • Description

  • Application Data

Description

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.

See full description

Application Data

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
Material Safety Data Sheet (MSDS)

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.

Anti-Frataxin antibody

Cat No.Pack SizePriceQty.
20ug£50.00
Bulk Quote