FEATURES

FAMILY

DESCRIPTION

Also known as ISCU_HUMAN, ISCU, NIFUN. Mitochondrial scaffold protein, of the core iron-sulfur cluster (ISC) assembly complex, that provides the structural architecture on which the [2Fe-2S] clusters are assembled (PubMed:34824239). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (PubMed:30031876, PubMed:34824239, PubMed:24971490, PubMed:29576242) (Probable). Exists as two slow interchanging conformational states, a structured (S) and disordered (D) form (PubMed:23940031). May modulate NFS1 desulfurase activity in a zinc-dependent manner (PubMed:30031876). Modulates the interaction between FXN and the cysteine desulfurase complex (PubMed:29576242)., Cytoplasmic scaffold protein, of the cytoplasmic core iron-sulfur cluster (ISC) assembly complex that provides the structural architecture on which the Fe-S clusters are assembled and may be involved in the cytoplasmic iron-sulfur protein biogenesis. Homodimer; Tyr-35-mediated dimerization of two iron- and sulfide-containing ISCU subunit bind to the cysteine desulfurase complex (PubMed:31101807, PubMed:34824239). Component of the mitochondrial core iron-sulfur cluster (ISC) complex composed of NFS1, LYRM4, NDUFAB1, ISCU, FXN, and FDX2; this complex is an heterohexamer containing two copies of each monomer (PubMed:21298097, PubMed:31101807) (Probable). Interacts (D-state) with NFS1 (homodimer form); each monomer interacts with the C-terminal regions of each NFS1 monomer (PubMed:23940031, PubMed:11060020). Interacts (monomer form) with FXN (via ferrous form); the interaction is possible when both are bound to the dimeric form of the cysteine desulfurase complex (NFS1:LYRM4) and enhances FXN interaction to the dimeric form of the cysteine desulfurase complex (NFS1:LYRM4) (PubMed:31101807, PubMed:29576242). Interacts with GLRX5 (PubMed:26100117). Interacts (D-state) with HSPA9 (PubMed:23940031, PubMed:26702583). Interacts (S-state) with HSCB; this interaction stimulates the ATPase activity of HSPA9 (PubMed:23940031, PubMed:20668094).

EXTERNAL LINKS

ISCU

Iron-sulfur cluster assembly enzyme ISCU

ISCU

Molecular Synopsis