Recombinant Human NFU1 Iron-Sulfur Cluster Scaffold Homolog Mitochondrial/NFU1
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novoprotein
Catalog#C873
SourceHEK293
DescriptionRecombinant Human NFU1 Iron-Sulfur Cluster Scaffold Homolog Mitochondrial/NFU1 is produced by our mammalian expression system in human cells. The target protein is expressed with sequence (Gly10-Pro254) of Human NFU1 fused with a polyhistidine tag at the C-terminus.
NamesNFU1 Iron-Sulfur Cluster Scaffold Homolog Mitochondrial, HIRA-Interacting Protein 5, NFU1, HIRIP5
Accession #Q9UMS0
ShippingThe product is shipped on dry ice/ice packs.
PurityGreater than 95% as determined by SEC-HPLC and reducing SDS-PAGE.
EndotoxinLess than 0.1 ng/μg (1 IEU/μg).
Amino Acid Sequence
GAAAVAAGLRRRFCHMLKNPYTIKKQPLHQFVQRPLFPLPAAFYHPVRYMFIQTQDTPNP NSLK FIPGKPVLETRTMDFPTPAAAFRSPLARQLFRIEGVKSVFFGPDFITVTKENEELD WNLLKPDI YATIMDFFASGLPLVTEETPSGEAGSEEDDEVVAMIKELLDTRIRPTVQEDGGDVIYKGFEDGIV QLKLQGSCTSCPSSIITLKNGIQNMLQFYIPEVEGVEQVMDDESDEKEANSPVDHHHHHH
BackgroundNFU1 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.