Molecular mechanism of iron-sulfur protein biogenesis in eukaryotes

Iron-sulfur (Fe/S) clusters serve as ancient cofactors of proteins with a function in catalysis, electron transport and the regulation of gene expression. The synthesis of a Fe/S cluster and its insertion into target proteins is a complex process which in eukaryotes requires more than 30 proteins in mitochondria and cytosol. These components were analyzed both in vivo and in vitro for their molecular function and a mechanistic model of Fe/S protein biogenesis was proposed. In humans, malfunctions in this pathway cause numerous diseases.