Molecular Aspects of Iron-Sulfur Protein Biogenesis

Three machineries have been identified to be responsible for the maturation of Fe-S cluster proteins in eukaryotes, (i) the mitochondrial Fe-S cluster assembly machinery, (ii) the mitochondrial inter-membrane exporting machinery and (iii) the cytosolic Fe-S protein machinery. Recently, the correlation of mutations in genes with pathogenic symptoms and diseases, gave rise to the determination and taxonomy of a novel syndrome, the Multiple Mitochondrial Dysfunction Syndrome (MMDS), which is inextricably linked with protein partners of the late steps of the mitochondrial Fe-S cluster assembly pathway. This PhD thesis elucidates the structure and the functional role of the human proteins NFU1, IBA57 and ISCA2 and illuminates several functional aspects of GLRX5, BOLA3 and ISCA1.