Mitochondria are essential in most eukaryotes and are involved in numerous biological
functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and …

Mitochondrial diseases are a group of genetic disorders that are characterized by defects in
oxidative phosphorylation and caused by mutations in genes in the nuclear DNA (nDNA) …

The essential process of iron-sulfur (Fe/S) cluster assembly (ISC) in mitochondria occurs in
three major phases. First,[2Fe-2S] clusters are synthesized on the scaffold protein ISCU2; …

Cellular iron homeostasis and mitochondrial iron homeostasis are interdependent.
Mitochondria must import iron to form iron–sulfur clusters and heme, and to incorporate …

Iron-sulfur (Fe-S) clusters are essential for many cellular processes, ranging from aerobic
respiration, metabolite biosynthesis, ribosome assembly and DNA repair. Mutations in NFU1 …

The recently solved crystal structures of the human cysteine desulfurase NFS1, in complex
with the LYR protein ISD11, the acyl carrier protein ACP, and the main scaffold ISCU, have …

Iron–sulfur clusters (Fe–S) are one of the most ancient, ubiquitous and versatile classes of
metal cofactors found in nature. Proteins that contain Fe–S clusters constitute one of the …

Disorders of the mitochondrial energy metabolism are clinically and genetically
heterogeneous. An increasingly recognized subgroup is caused by defective mitochondrial …

Mammalian A-type proteins, ISCA1 and ISCA2, are evolutionarily conserved proteins
involved in iron–sulfur cluster (Fe–S) biogenesis. Recently, it was shown that ISCA1 and …

Iron–sulfur (Fe-S) clusters (ISCs) are ubiquitous cofactors essential to numerous
fundamental cellular processes. Assembly of ISCs and their insertion into apoproteins …