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NFU1 is a protein that is localized to mitochondria and plays a critical role in iron-sulfur cluster biogenesis. The encoded protein assembles and transfers 4Fe-4S clusters to target apoproteins including succinate dehydrogenase and lipoic acid synthase. Mutations in this gene are a cause of multiple mitochondrial dysfunctions syndrome-1, and pseudogenes of this gene are located on the short arms of chromosomes 1 and 3. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.
Gene Name: | NFU1 Iron-sulfur Cluster Scaffold Homolog (S. Cerevisiae) |
Synonyms: | NFU1, CGI-33, HIRA-interacting protein 5, HIRA interacting protein 5, MMDS1, NIFUC, Nfu, NIFU, HIRIP, HIRIP5 |
Target Sequences: | NM_015700 NP_056515.2 Q9UMS0 |
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