Mitochondrial De Novo Assembly of Iron–Sulfur Clusters in Mammals: Complex Matters in a Complex That Matters

Iron–sulfur clusters (Fe–S or ISC) are essential cofactors that function in a wide range of biological pathways. In mammalian cells, Fe–S biosynthesis primarily relies on mitochondria and involves concerted group evolutionary-conserved proteins forming the ISC pathway. early stage pathway, core complex is required for de novo assembly Fe–S. humans, comprises cysteine desulfurase NFS1, scaffold protein ISCU2, frataxin (FXN), ferredoxin FDX2, regulatory/accessory ISD11 Acyl Carrier Protein (ACP). recent years, field has made significant advances unraveling structure mechanism underlying its function. Herein, we review key findings related to components. We highlight some unanswered questions provide model within complex. addition, briefly touch genetic diseases associated with mutations