LYRM4 Antibody

1. analysis of the NFS1-ISD11-ACP (SDA) complex forms the core of the iron-sulfur (Fe-S) assembly complex and associates with assembly proteins ISCU2, frataxin (FXN), and ferredoxin to synthesize Fe-S clusters PMID: 28634302
2. Human Isd11 (ISD11) is a helical protein which exists in solution as an equilibrium between monomer, dimeric and tetrameric species when in the absence of human Nfs1 (NFS1). Recombinant ISD11 expressed in E. coli co-purifies with the bacterial orthologue of NFS1, IscS. PMID: 27427956
3. The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation. PMID: 28271877
4. Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components. PMID: 27519411
5. Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1, and thereby fails to prevent NFS1 aggregation resulting in impairment of the Fe-S cluster biogenesis PMID: 26342079
6. Homozygous mutation in LYRM4 was reported in two patients with combined oxidative phosphorylation deficiency. PMID: 23814038
7. LYRM4 downregulation may be one of the mechanisms involved in inefficient oxidative phosphorylation and oxidative stress, increasingly recognised as contributors to schizophrenia pathogenesis. PMID: 21968932
8. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
9. Frataxin interacts with ISD11 and multiple mitochondrial chaperones. PMID: 17331979
10. ISD11 is important in the biogenesis of Fe-S clusters in mammalian cells. PMID: 19454487

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HGNC: 21365

OMIM: 613311

KEGG: hsa:57128

STRING: 9606.ENSP00000443900

UniGene: Hs.387755