Synonyms:CGI-33, ENTREZ:27247, HGNC:16287, HIRIP, HIRIP5, MIM:608100, MMDS1, NFU1 iron-sulfur cluster scaffold, NFU1, NIFUC, NM_001002755, NM_001002756, NM_001002757, NM_001374284, NM_015700, NP_001002755, NP_001002756, NP_001361213, NP_056515, NR_045631, NR_045632, Nfu, NifU
Omim:OMIM:608100
Id:2e3384d1-1db1-58ac-ac24-013dea8d6933
Hgnc:HGNC:16287
Description:NFU1 iron-sulfur cluster scaffold
This gene encodes 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. [provided by RefSeq, Dec 2011]