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Required for nuclear and mitochondrial iron-sulfur protein biosynthesis (By similarity). Additionally we are shipping LYR Motif Containing 4 Antibodies (25) and LYR Motif Containing 4 Kits (11) and many more products for this protein.
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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 (show NFS1 Proteins)). Recombinant ISD11 expressed in E. coli co-purifies with the bacterial orthologue of NFS1 (show NFS1 Proteins), IscS (show NFS1 Proteins).
The NFS1 (show NFS1 Proteins)/ISD11 complex further interacts with scaffold protein (show HOMER1 Proteins) ISCU (show ISCU Proteins) and regulator protein frataxin (show FXN Proteins), thereby forming a quaternary complex for Fe-S cluster formation.
Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN (show FXN Proteins)(42-210)]24.[NFS1 (show NFS1 Proteins)]24.[ISD11]24.[ISCU (show ISCU Proteins)]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components.
Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1 (show NFS1 Proteins), and thereby fails to prevent NFS1 (show NFS1 Proteins) aggregation resulting in impairment of the Fe-S cluster biogenesis
Homozygous mutation in LYRM4 was reported in two patients with combined oxidative phosphorylation deficiency.
LYRM4 downregulation may be one of the mechanisms involved in inefficient oxidative phosphorylation and oxidative stress, increasingly recognised as contributors to schizophrenia pathogenesis.
Frataxin (show FXN Proteins) interacts with ISD11 and multiple mitochondrial chaperones.
ISD11 is important in the biogenesis of Fe-S clusters in mammalian cells.
Required for nuclear and mitochondrial iron-sulfur protein biosynthesis (By similarity).
LYR motif-containing protein 4
, LYR motif containing 4
, homolog of yeast Isd11
, mitochondrial matrix Nfs1 interacting protein