Tissue Specificity | Detected in heart, liver, skeletal muscle, brain, pancreas, kidney, lung and placenta. |
Post Translational Modifications | Phosphorylation at Ser-14 is required for ISCU protein stabilization in the cytosol, whereas dephosphorylation of Ser-14, due to the inhibition of mTORC1 (mammalian target of rapamycin complex 1) complex, leads to degradation of the precursor form and ultimately to a decrease in the mitochondrial mature form. Cysteine persulfide is reduced by thiol-containing molecules such as glutathione and L-cysteine. |
Function | 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. |
Protein Name | Iron-Sulfur Cluster Assembly Enzyme IscuNifu-Like N-Terminal Domain-Containing ProteinNifu-Like Protein |
Database Links | Reactome: R-HSA-1362409 Q9H1K1-1Reactome: R-HSA-9694301Reactome: R-HSA-9854311 Q9H1K1-1Reactome: R-HSA-9865881 Q9H1K1-1 |
Cellular Localisation | Isoform 1: MitochondrionIsoform 2: CytoplasmNucleus |
Alternative Antibody Names | Anti-Iron-Sulfur Cluster Assembly Enzyme Iscu antibodyAnti-Nifu-Like N-Terminal Domain-Containing Protein antibodyAnti-Nifu-Like Protein antibodyAnti-ISCU antibodyAnti-NIFUN antibody |
Information sourced from Uniprot.org