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Required for nuclear and mitochondrial iron-sulfur protein biosynthesis (By similarity). Zusätzlich bieten wir Ihnen LYR Motif Containing 4 Kits (11) und LYR Motif Containing 4 Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.
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Human Polyclonal LYRM4 Primary Antibody für IHC, IHC (p) - ABIN4894050
Lim, Friemel, Marum, Tucker, Bruno, Riley, Christodoulou, Kirk, Boneh, DeGennaro, Springer, Mootha, Rouault, Leimkühler, Thorburn, Compton: Mutations in LYRM4, encoding iron-sulfur cluster biogenesis factor ISD11, cause deficiency of multiple respiratory chain complexes. in Human molecular genetics 2013
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
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.
The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, 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(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]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, and thereby fails to prevent NFS1 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.
Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients
Frataxin 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