Mutant is deficient in mitochondrial iron transport activity; mitochondrial iron proteins are decreased; TTP/TTP homoexchange is preserved but TTP/TMP heteroexchange is disrupted, perhaps because proton coupling is dependent on the E248 acidic residue; rate of Fe-S cluster synthesis in isolated and intact mitochondria is decreased
List of references used specifically to curate the information in Overview section.
Knight SAB, et al. (2019) Splitting the functions of Rim2, a mitochondrial iron/pyrimidine carrier. Mitochondrion 47:256-265