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Human Polyclonal NFS1 Primary Antibody for WB - ABIN522493
Huang, Becker, Whitnall, Suryo Rahmanto, Ponka, Richardson: Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant. in Proceedings of the National Academy of Sciences of the United States of America 2009
AtFH binds and modulates Nfs1 kinetics in mitochondria.
NFS1 has a role in mediating the assembly of iron-sulfur clusters in mitochondria.
FXN binds the NFS1-ISD11-ACP-ISCU complex (SDAU), to activate the desulfurase activity and Fe-S cluster biosynthesis. In the absence of FXN, the NFS1-ISD11-ACP (SDA) complex was reportedly inhibited by binding of recombinant ISCU
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
NFS1 maintains the iron sulfur clusters in proteins that are essential for protecting them from oxidative damage; inactivating NFS1 or iron sulfur clusters can trigger ferroptosis, a non-apoptotic form of cell death, in cancer cells
human NFS1 was almost fully able to complement the role of IscS in Moco biosynthesis when its specific interaction partner protein MOCS3 from humans was also present.
The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation.
FDX1 and FDX2 both bind NFS1 and donate electrons for iron-sulfur cluster biosynthesis.
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
The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity.
NFS1 binds preferentially to the D-state of ISCU while mtHSP70 binds preferentially to the D-state of ISCU and HSC20 binds preferentially to the S-state of ISCU.
the interaction of NFS1 and MOCS3 in the cytosol of human cells, is reported.
Nfs1, the cysteine desulfurase responsible for providing sulfur for cluster formation, is required for the increased Isu stability occurring after disruption of cluster formation on or transfer from Isu
the cytosolic form of ISCS is a functional cysteine desulfurase that can collaborate with cytosolic ISCU to promote de novo iron-sulfur cluster formation
Results show that human Nfs1 is required inside mitochondria for efficient maturation of cellular iron/sulfur proteins.
Nfs1 acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis
Icp55 protease and its substrate Nfs1 appear to be dual distributed between the nucleus and mitochondria
there is a mechanism that primarily dedicates m-Nfs1 to the biogenesis of mitochondrial Fe-S clusters in order to maintain cell survival
While IFN-gamma alone induced Nfs1 protein instability, LPS triggered a delayed decline of Nfs1, rather involving transcriptional events or mRNA instability.
Iron-sulfur clusters are required for the function of many cellular enzymes. The proteins encoded by this gene supply inorganic sulfur to these clusters by removing the sulfur from cysteine, creating alanine in the process. This gene uses alternate in-frame translation initiation sites to generate mitochondrial forms and cytoplasmic/nuclear forms. Selection of the alternative initiation sites is determined by the cytosolic pH. The encoded proteins belong to the class-V family of pyridoxal phosphate-dependent aminotransferases. Alternatively spliced transcript variants have been described.
cysteine desulfurase NFS1
, cysteine desulfurase, (m-Nfs1)
, cysteine desulfurase
, NFS1 nitrogen fixation 1 homolog
, cysteine desulfurase, mitochondrial
, nitrogen fixation 1 (S. cerevisiae, homolog)
, nitrogen-fixing bacteria S-like protein
, nitrogen fixation gene 1
, NFS1 nitrogen fixation 1 homolog (S. cerevisiae)
, nifS-like (sic)
, nitrogen fixation gene, yeast homolog 1
, LOW QUALITY PROTEIN: cysteine desulfurase, mitochondrial
, NFS1 nitrogen fixation 1