Iron-sulfur-protein biogenesis in eukaryotes.
Iron-sulfur (Fe-S) clusters (ISCs) are versatile, ancient co-factors of proteins that are involved in electron transport, enzyme catalysis and regulation of gene expression. The synthesis of ISCs and their insertion into apoproteins involves the function of complex cellular machineries. In eukaryotes, the mitochondrial ISC-assembly machinery is involved in the maturation of all cellular iron-sulfur proteins. A mitochondrial export machinery and a recently discovered cytosolic assembly system specifically participate in the maturation of cytosolic and nuclear iron-sulfur proteins. Of the approximately 20 assembly components, more than ten are encoded by essential genes, which indicates that the process is indispensable for life. Mutations in two of the assembly components lead to neurological diseases. The essential character of Fe-S-protein biogenesis in eukaryotes and its importance for human disease identifies this evolutionary ancient process as one of the most important biosynthetic pathways of life.
Lill
Roland
R
Institut für Zytobiologie und Zytopathologie, Philipps-Universität Marburg, Robert-Koch-Strasse 6, D-35033 Marburg, Germany. Lill@mailer.uni-marburg.de
Mühlenhoff
Ulrich
U
D000818:Animals; D001419:Bacteria / Q000235:genetics / Q000378:metabolism; D005075:Biological Evolution; D002467:Cell Nucleus / Q000378:metabolism; D003600:Cytosol / Q000378:metabolism; D005057:Eukaryotic Cells; D006801:Humans; D007506:Iron-Sulfur Proteins / Q000096:biosynthesis* / Q000235:genetics; D008928:Mitochondria / Q000378:metabolism; D008954:Models, Biological; D009154:Mutation
D007506:Iron-Sulfur Proteins