UniprotKB/SwissProt ID: O49627 (O49627)
Gene Name:
ISU1
Organism: Arabidopsis thaliana (Mouse-ear cress)
Function: Scaffold protein for the de novo synthesis of iron-sulfur (Fe-S) clusters within mitochondria, which is required for maturation of both mitochondrial and cytoplasmic [2Fe-2S] and [4Fe-4S] proteins (PubMed:15507320, PubMed:17417719). First, a [2Fe-2S] cluster is transiently assembled on the scaffold protein ISCU (ISU1, ISU2 or ISU3). In a second step, the cluster is released from ISCU, transferred to a glutaredoxin, followed by the formation of mitochondrial [2Fe-2S] proteins, the synthesis of [4Fe-4S] clusters and their target-specific insertion into the recipient apoproteins. Cluster assembly on ISCU depends on the function of the cysteine desulfurase complex NFS1-ISD11, which serves as the sulfur donor for cluster synthesis, the iron-binding protein frataxin as the putative iron donor, and the electron transfer chain comprised of ferredoxin reductase and ferredoxin, which receive their electrons from NADH (By similarity)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion matrix. Cytoplasm, cytosol
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