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Overview

Uniprot ID	Q9D7P6
Protein Name	Iron-sulfur cluster assembly enzyme ISCU
Gene Name	Iscu
Organism	Mus musculus

Kla Sites from experimental identification

Position	Flanking peptide
39	PARLYHKKVVDHYEN
83	LQIQVDEKGKIVDAR

Function

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. 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

Protein Sequence

10 MAAATGAGRL 20 RRAASALLLR 30 SPRLPARELS 40 APARLYHKKV 50 VDHYENPRNV 60 GSLDKTSKNV 70 GTGLVGAPAC 80 GDVMKLQIQV 90 DEKGKIVDAR 100 FKTFGCGSAI 110 ASSSLATEWV 120 KGKTVEEALT 130 IKNTDIAKEL 140 CLPPVKLHCS 150 MLAEDAIKAA 160 LADYKLKQES KKEEPEKQ

Gene Ontology

Classification	GO ID	Description
Cellular Component	GO:0005737	cytoplasm
Cellular Component	GO:0005829	cytosol
Cellular Component	GO:0099128	mitochondrial [2Fe-2S] assembly complex
Cellular Component	GO:0005759	mitochondrial matrix
Cellular Component	GO:0005739	mitochondrion
Molecular Function	GO:0051537	2 iron, 2 sulfur cluster binding
Molecular Function	GO:0008198	ferrous iron binding
Molecular Function	GO:0140132	iron-sulfur cluster chaperone activity
Molecular Function	GO:0060090	molecular adaptor activity
Molecular Function	GO:0042803	protein homodimerization activity
Molecular Function	GO:0008270	zinc ion binding
Biological Process	GO:0044571	[2Fe-2S] cluster assembly
Biological Process	GO:0044572	[4Fe-4S] cluster assembly
Biological Process	GO:0006879	intracellular iron ion homeostasis
Biological Process	GO:0016226	iron-sulfur cluster assembly
Biological Process	GO:1904439	negative regulation of iron ion import across plasma membrane
Biological Process	GO:1902958	positive regulation of mitochondrial electron transport, NADH to ubiquinone

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.