BOLA3 and Fatal multiple mitochondrial dysfunction syndrome type 2: In particular, mutations in the genes encoding for the proteins acting late in the ISC machinery and responsible for the maturation of [4Fe-4S] proteins, have been connected to a group of autosomal recessive mitochondrial energy metabolism disorders, called multiple mitochondrial dysfunctions syndromes (MMDSs) types 1 to 5, associated with NFU1, BOLA3, IBA57, ISCA2, and ISCA1, respectively [11,12,13,14,15].