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Proteins/UNIPROT:Q9H1K1
[Isoform 1]: 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. 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 (Probable). Exists as two slow interchanging conformational states, a structured (S) and disordered (D) form. May modulate NFS1 desulfurase activity in a zinc-dependent manner. Modulates the interaction between FXN and the cysteine desulfurase complex..
[Isoform 2]: 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..
Function from UniProtKB (Q9H1K1), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 2 · substrate of
2 compounds
2 compounds.
0 reported routes2 assembled routes
2 rows
28 statements
28 statements · 5 reference databases · Subject: ISCU · Sources: gene ontology, Hetionet, Human-GEM, Reactome, WikiPathways
1 reaction
2L-cysteine+2iron(2+)+NADPH(4-)2L-alanine+proton+NADP(3-)+di-mu-sulfido-diiron(2+)
Catalysed by NDUFAB1 & FDXR & FXN & FDX2 & ISCU & LYRM4 & NFS1
Reaction pageLeft to rightBalancedmitochondrial matrix
Human-GEM MAR20185CC BY 4.0Reference data
3 names
Resolves to: ISCU