Description
NFU1 Iron-Sulfur Cluster Scaffold Homolog Mitochondrial (NFU1) belongs to the nifU family. NFU1 is widely expressed in many tissues, but expression in adult lung is weak compared to fetal lung. NFU1 interacts with HIRA and EPM2A/laforin which can assemble [4Fe-2S] clusters and deliver them to target proteins. Defects in NFU1 are the cause of multiple mitochondrial dysfunctions syndrome type 1 (MMDS1) which is a severe disorder of systemic energy metabolism, resulting in respiratory failure, lack of neurologic development, weakness, lactic acidosis and early death