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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.
analysis of the NFS1-ISD11 (show LYRM4 Antibodies)-ACP (show NDUFAB1 Antibodies) (SDA) complex forms the core of the iron-sulfur (Fe-S) assembly complex and associates with assembly proteins ISCU2 (show ISCU Antibodies), frataxin (FXN (show FXN Antibodies)), 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 (show MOCS3 Antibodies) from humans was also present.
The NFS1/ISD11 (show LYRM4 Antibodies) complex further interacts with scaffold protein (show HOMER1 Antibodies) ISCU (show ISCU Antibodies) and regulator protein frataxin (show FXN Antibodies), thereby forming a quaternary complex for Fe-S cluster formation.
FDX1 (show FDX1 Antibodies) 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 (show FXN Antibodies)(42-210)]24.[NFS1]24.[ISD11 (show LYRM4 Antibodies)]24.[ISCU (show ISCU Antibodies)]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components.
Our findings highlight that the ISD11 (show LYRM4 Antibodies) 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 (show FXN Antibodies) effects on Nfs1, explaining the bypassing activity.
NFS1 binds preferentially to the D-state of ISCU (show ISCU Antibodies) while mtHSP70 (show HSPA9 Antibodies) binds preferentially to the D-state of ISCU (show ISCU Antibodies) and HSC20 (show HSCB Antibodies) binds preferentially to the S-state of ISCU (show ISCU Antibodies).
the interaction of NFS1 and MOCS3 (show MOCS3 Antibodies) in the cytosol of human cells, is reported.
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 (show IFNG Antibodies) alone induced Nfs1 protein instability, LPS (show TLR4 Antibodies) 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