2y5c: Difference between revisions
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[https://www.uniprot.org/uniprot/FDX2_HUMAN FDX2_HUMAN] The disease is caused by variants affecting the gene represented in this entry. | [https://www.uniprot.org/uniprot/FDX2_HUMAN FDX2_HUMAN] The disease is caused by variants affecting the gene represented in this entry. | ||
== Function == | == Function == | ||
[https://www.uniprot.org/uniprot/FDX2_HUMAN FDX2_HUMAN] Electron donor, of the core iron-sulfur cluster (ISC) assembly complex, that acts to reduce the persulfide into sulfide during [2Fe-2S] clusters assembly on the scaffolding protein ISCU (PubMed:28001042). 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 (By similarity).[UniProtKB:Q9H1K1]<ref>PMID:28001042</ref> | [https://www.uniprot.org/uniprot/FDX2_HUMAN FDX2_HUMAN] Electron donor, of the core iron-sulfur cluster (ISC) assembly complex, that acts to reduce the persulfide into sulfide during [2Fe-2S] clusters assembly on the scaffolding protein ISCU (PubMed:28001042). 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 (By similarity). 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 (By similarity). Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly (By similarity). Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity). Essential for coenzyme Q biosynthesis: together with FDXR, transfers the electrons required for the hydroxylation reaction performed by COQ6 (PubMed:38425362).[UniProtKB:Q9H1K1]<ref>PMID:28001042</ref> <ref>PMID:38425362</ref> | ||
== References == | == References == | ||
<references/> | <references/> | ||
