LYRM4


Description

The LYRM4 (LYR motif containing 4) is a protein-coding gene located on chromosome 6.

LYRM4 is a protein that plays a crucial role in the assembly of iron-sulfur clusters, essential components of many enzymes. It is a stabilizing factor in the core iron-sulfur cluster (ISC) assembly complex, working in conjunction with NDUFAB1 to maintain the stability and activity of NFS1, an enzyme critical for the production of iron-sulfur clusters. LYRM4 also participates in the assembly of these clusters onto a scaffolding protein called ISCU. This complex is found in mitochondria, where it is involved in the synthesis of new iron-sulfur clusters. LYRM4 is also known to interact with the cytoplasmic form of NFS1, suggesting a potential role in iron-sulfur cluster biogenesis outside of the mitochondria. In addition to its role in the ISC complex, LYRM4 forms a dimer and is found in a larger complex that includes NFS1, FXN, and ISCU.

LYRM4 is a stabilizing factor in the core iron-sulfur cluster (ISC) assembly complex, where it works alongside NDUFAB1 to regulate the stability and cysteine desulfurase activity of NFS1. It also plays a role in the assembly of [2Fe-2S] clusters on the scaffolding protein ISCU. The ISC complex is involved in the de novo synthesis of [2Fe-2S] clusters, a critical step in mitochondrial iron-sulfur protein biogenesis. This process begins with the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1), which produces persulfide, delivered to the scaffold protein ISCU in a FXN-dependent manner. FDX2 stabilizes the complex by providing reducing equivalents for [2Fe-2S] cluster assembly. The cluster is then transferred from ISCU to chaperone proteins like HSCB, HSPA9, and GLRX5. LYRM4 may also participate in iron-sulfur protein biogenesis in the cytoplasm through its interaction with the cytoplasmic form of NFS1.

LYRM4 is also known as C6orf149, CGI-203, COXPD19, ISD11.

Associated Diseases


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