Mouse Frataxin (FXN) ELISA Kit-Sandwich

Frataxin, located in the mitochondria, acts as an activator for persulfide transfer to the scaffold protein ISCU, playing a key role in the core iron-sulfur cluster (ISC) assembly complex and participating in [2Fe-2S] cluster assembly. In mouse models, the absence of frataxin results in embryonic lethality, highlighting its crucial role in embryonic development. Research using mouse models is instrumental in understanding frataxin's function. For instance, the phenotype of Friedreich's ataxia (FA) can be replicated through gene knockout or mouse models expressing frataxin mutants. The expression and function of frataxin vary across different tissues. In the heart, liver, and skeletal muscle, frataxin deficiency leads to metabolic disorders and dysfunction within those specific tissues.