Affinage

NFU1

NFU1 iron-sulfur cluster scaffold homolog, mitochondrial · UniProt Q9UMS0

Length
254 aa
Mass
28.5 kDa
Annotated
2026-04-29
59 papers in source corpus 14 papers cited in narrative 14 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

NFU1 is a mitochondrial late-acting iron-sulfur cluster carrier that receives [2Fe-2S] clusters sequentially from ISCU2 and ISCA1, reductively assembles them into a bridging [4Fe-4S] cluster coordinated by its C-terminal CXXC motif between two NFU1 subunits, and delivers this cluster to a specific subset of mitochondrial client proteins (PMID:22077971, PMID:27818104, PMID:32776106, PMID:33711344). Its most critical client is lipoic acid synthase (LIAS), whose auxiliary [4Fe-4S] cluster is regenerated by NFU1 during catalytic turnover to enable multi-round lipoylation of pyruvate dehydrogenase, α-ketoglutarate dehydrogenase, and the glycine cleavage complex; additional clients include succinate dehydrogenase (complex II) and the mitoribosome assembly factor METTL17 (PMID:36281303, PMID:21944046, PMID:37823603). The N-terminal domain modulates partner recognition during cluster transfer through structural plasticity, while ISCA1 orchestrates a transient ISCA1-ISCA2-NFU1 ternary complex that channels [4Fe-4S] clusters to the ISCA1-NFU1 delivery intermediate (PMID:37211204, PMID:33711344). Pathogenic missense mutations (e.g., Gly208Cys, Gly189Arg) cause multiple mitochondrial dysfunctions syndrome 1 (MMDS1) by disrupting NFU1 dimerization or stability and thereby abolishing cluster acquisition (PMID:28161430, PMID:28906594).

Mechanistic history

Synthesis pass · year-by-year structured walk · 9 steps
  1. 2011 High

    Establishing that NFU1 is not a general Fe-S scaffold but a specialized late-acting maturation factor for a restricted subset of mitochondrial [4Fe-4S] clients — primarily lipoic acid synthase and SDH — resolved its position in the Fe-S biogenesis pathway and linked its loss to defective lipoylation.

    Evidence RNAi in human cells and yeast deletion mutants with enzymatic activity assays for LAS/PDHC/SDH, plus isoform-specific retroviral complementation in patient fibroblasts

    PMID:21944046 PMID:22077971

    Open questions at the time
    • Whether NFU1 directly binds and transfers a [4Fe-4S] cluster was not demonstrated
    • The donor(s) of the cluster to NFU1 were unknown
    • Full client repertoire beyond LAS and SDH was not defined
  2. 2016 High

    Structural determination revealed that NFU1's C-terminal CXXC domain coordinates a bridging [4Fe-4S] cluster between two subunits forming a cluster-linked dimer, and that holo-NFU1 can activate apo-aconitase, proving its cluster-transfer competence in vitro.

    Evidence NMR structure of N- and C-terminal domains, SAXS, SEC, and in vitro aconitase activation assay; yeast genetic and proteomic interaction mapping with ISA complex and client proteins

    PMID:27532773 PMID:27818104

    Open questions at the time
    • The physiological cluster donor to NFU1 remained unidentified
    • Whether NFU1 directly interacts with LIAS was not tested
    • The role of the N-terminal domain in partner selectivity was unclear
  3. 2017 High

    Biochemical dissection of MMDS1-causing mutations (Gly208Cys and Gly189Arg) showed they impair cluster acquisition not by altering the cluster-binding cysteines but by disrupting dimerization and structural integrity, establishing the mechanistic basis of disease pathogenesis.

    Evidence In vitro SEC, CD spectroscopy, Fe-S reconstitution assays, and site-directed mutagenesis panels for both pathogenic variants

    PMID:28161430 PMID:28906594

    Open questions at the time
    • Whether these mutations also impair downstream cluster transfer to clients was not directly tested
    • Impact on the full client repertoire in patient cells was not assessed
    • G189R finding from a single lab (Medium confidence individually)
  4. 2020 High

    Identification of ISCU2 and ISCA1 as sequential [2Fe-2S] cluster donors to NFU1, with ferredoxin 2-assisted reductive coupling into a [4Fe-4S] cluster, defined the complete upstream delivery pathway and mapped the interaction site to a conserved hydrophobic patch on NFU1's C-terminal α-helix.

    Evidence NMR interaction mapping, ITC (Kd ~1.1 µM), in vitro Fe-S cluster transfer assays, site-directed mutagenesis abolishing cluster acquisition and downstream LIAS-dependent lipoylation in human cells

    PMID:32151725 PMID:32776106

    Open questions at the time
    • The reductive coupling mechanism and precise role of ferredoxin 2 were not structurally resolved
    • Whether ISCU transfers a [4Fe-4S] or two sequential [2Fe-2S] clusters was debated between the two studies
  5. 2021 High

    NMR-based structural characterization showed that ISCA1 orchestrates a transient ISCA1-ISCA2-NFU1 ternary complex that channels the [4Fe-4S] cluster from the ISCA1-ISCA2 assembly site to an ISCA1-NFU1 intermediate, clarifying the handoff mechanism in the late biogenesis pathway.

    Evidence NMR interaction mapping and structural modeling of binary and ternary apo/holo complexes, in vitro cluster transfer assays

    PMID:33711344

    Open questions at the time
    • Kinetics and directionality of cluster transfer within the ternary complex were not quantified
    • Role of ferredoxin 2 in the ternary complex context was not addressed
  6. 2022 High

    In vitro reconstitution demonstrated that NFU1 forms a tight complex with LIAS and regenerates its auxiliary [4Fe-4S] cluster during catalytic turnover, enabling multiple rounds of lipoyl cofactor synthesis — establishing NFU1 as a catalytic partner rather than a one-shot donor.

    Evidence Radical SAM activity assay for LIAS with NFU1, protein complex formation assay, Fe-S cluster transfer assay; BOLA3 shown to have no direct effect

    PMID:36281303

    Open questions at the time
    • Structural basis of the NFU1-LIAS complex is not resolved
    • Whether multi-turnover regeneration occurs in cellulo was not shown
    • Role of BOLA3 in vivo remains unexplained despite no in vitro effect
  7. 2023 Medium

    Discovery that NFU1 delivers a [4Fe-4S] cluster to METTL17 expanded the client repertoire beyond metabolic enzymes to mitoribosome assembly, revealing a previously unrecognized role in mitochondrial translation that explains some NFU1-deficiency phenotypes.

    Evidence siRNA silencing of Fe-S biogenesis factors, mitoribosome assembly and mitochondrial translation assays, NFU1-patient fibroblast analysis

    PMID:37823603

    Open questions at the time
    • Direct physical interaction between NFU1 and METTL17 was not reconstituted in vitro
    • Quantitative contribution of translation defects versus lipoylation defects to MMDS1 pathology is unknown
  8. 2023 High

    SAXS and paramagnetic NMR structural models of the ISCA1-ISCA2-NFU1 assembly intermediates revealed that NFU1's N-terminal domain undergoes structural rearrangements that modulate partner recognition and cluster transfer, assigning a functional role to the previously enigmatic N-terminal domain.

    Evidence SEC-SAXS, paramagnetic NMR, structural modeling of apo and holo complexes

    PMID:37211204

    Open questions at the time
    • High-resolution atomic structure of the full holo-NFU1 in complex with clients is lacking
    • Whether N-terminal domain plasticity differs for different client proteins is untested
  9. 2023 Medium

    C. elegans studies showed that NFU1 orthologue loss-of-function affects neuromuscular junction acetylcholine signaling, with patient-specific alleles causing distinct pre- versus post-synaptic defects, extending the phenotypic impact of NFU1 deficiency to the nervous system.

    Evidence CRISPR knock-in of patient variants in C. elegans, acetylcholine sensitivity assays, genetic epistasis

    PMID:36645076

    Open questions at the time
    • The molecular link between mitochondrial Fe-S cluster deficiency and cholinergic signaling is unknown
    • Whether these neuromuscular phenotypes are conserved in mammals has not been tested

Open questions

Synthesis pass · forward-looking unresolved questions
  • Key unresolved questions include the complete client repertoire of NFU1 in human mitochondria, the high-resolution structural basis of NFU1-client recognition (especially NFU1-LIAS), the in vivo mechanism by which BOLA3 cooperates with NFU1 despite lacking direct in vitro effects on cluster transfer, and how NFU1 deficiency leads to neuromuscular phenotypes.
  • No high-resolution structure of an NFU1-client complex exists
  • BOLA3's in vivo role in NFU1-dependent cluster delivery is mechanistically unexplained
  • Full inventory of NFU1 client proteins in human cells has not been systematically determined

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140104 molecular carrier activity 4
Localization
GO:0005739 mitochondrion 4
Pathway
R-HSA-1430728 Metabolism 3 GO:0005739 mitochondrion 2 R-HSA-1643685 Disease 2
Partners
BOLA3FDX2ISCA1ISCA2ISCULIASMETTL17
Complex memberships
ISCA1-ISCA2-NFU1 ternary complexISCA1-NFU1 binary complexNFU1-LIAS complex

Evidence

Reading pass · 14 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2011 NFU1 functions as a late-acting maturation factor for a specific subset of mitochondrial Fe-S proteins. RNAi depletion of NFU1 in human cells markedly decreased lipoic acid synthase (LAS) activity and in turn pyruvate dehydrogenase complex (PDHC) activity, and reduced succinate dehydrogenase (SDH) amount, but did not affect other Fe-S proteins tested. By contrast, depletion of the general scaffold ISCU severely affected all tested Fe-S proteins, demonstrating that NFU1 has a specialized rather than general role in mitochondrial Fe-S cluster maturation. RNA interference in human cell culture, enzymatic activity assays, protein quantification; yeast NFU1 deletion with lipoylation and SDH activity measurements; yeast missense mutant functional complementation American journal of human genetics High 22077971
2011 Both NFU1 (mitochondrial isoform) and BOLA3 (isoform 1) are required for the maturation of lipoate-containing 2-oxoacid dehydrogenases and for respiratory chain complex assembly. Retroviral transduction with the mitochondrial isoform of NFU1, but not the cytosolic isoform, restored respiratory chain function and oxoacid dehydrogenase complex activity in patient fibroblasts. Retroviral complementation of patient fibroblast lines with isoform-specific NFU1 constructs; enzymatic activity assays for respiratory chain and oxoacid dehydrogenase complexes American journal of human genetics High 21944046
2016 Human mitochondrial NFU1 binds and transfers a [4Fe-4S] cluster. The C-terminal domain contains the two cysteines that coordinate one [4Fe-4S] cluster bridging two NFU1 subunits to form a cluster-linked dimer; three such dimers associate via their N-terminal domains to form hexameric holo-NFU1. Holo-NFU1 can activate apo-aconitase in vitro, confirming its cluster-transfer activity. NMR spectroscopy (3D structures of N- and C-terminal domains), small-angle X-ray scattering (SAXS), size-exclusion chromatography, in vitro aconitase activation assay Structure High 27818104
2016 Yeast Nfu1 functions in a late step of [4Fe-4S] cluster biogenesis and physically interacts with components of the ISA [4Fe-4S] assembly complex and with [4Fe-4S] client proteins. Bol3 (human BOLA3 ortholog) functions together with Nfu1 in this late step, whereas Bol1 functions earlier with the monothiol glutaredoxin Grx5. Genetic studies in yeast (deletion mutants, growth under oxidative metabolism), proteomic interaction studies (mass spectrometry-based), epistasis analysis eLife High 27532773
2020 Human ISCU (ISCU2) directly interacts with NFU1 (Kd ~1.1 µM as measured by ITC), and ISCU[4Fe-4S] transfers its Fe-S cluster to apo-NFU1 in the absence of a chaperone to form holo-NFU1. ISCU[2Fe-2S] does not transfer its cluster to apo-NFU1. The interaction site maps to the cluster-binding region of ISCU and two α-helices in the C-terminal domain of NFU1. NMR spectroscopy, SAXS, size-exclusion chromatography, isothermal titration calorimetry (ITC), in vitro Fe-S cluster transfer assay Journal of structural biology High 32151725
2020 ISCU2 and ISCA1 are the direct sequential donors of [2Fe-2S] clusters to mitochondrial NFU1, which dimerizes and reductively forms a bridging [4Fe-4S] cluster aided by ferredoxin 2. The interaction site maps to a conserved hydrophobic patch at the end of the C-terminal α-helix of NFU1; mutagenesis at this site abolishes cluster acquisition and downstream maturation of lipoic acid synthase (LIAS), thereby impairing lipoylation of PDH, α-KGDH, and glycine cleavage complex components. Genetic knockdown/knockout in human cells, biochemical pulldown/co-IP, site-directed mutagenesis of NFU1, Fe-S cluster transfer assays, enzymatic activity assays for LIAS-dependent lipoylation Human molecular genetics High 32776106
2021 ISCA1 is the key orchestrator of mitochondrial [4Fe-4S] protein maturation: it interacts with both NFU1 and ISCA2 (which do not interact with each other), promotes formation of a transient ISCA1-ISCA2-NFU1 ternary complex, and drives [4Fe-4S] cluster transfer from the ISCA1-ISCA2 assembly site to a cluster-binding site formed between ISCA1 and the C-terminal domain of NFU1. NMR spectroscopy (interaction mapping and structural modeling of complexes), in vitro Fe-S cluster transfer assays Journal of molecular biology High 33711344
2022 Human NFU1 forms a tight complex with human lipoyl synthase (LIAS) in vitro and efficiently restores the auxiliary [4Fe-4S] cluster of LIAS during catalytic turnover, enabling multiple rounds of lipoyl cofactor synthesis. BOLA3 has no direct effect on Fe-S cluster transfer from NFU1 to LIAS. In vitro reconstitution of LIAS activity in the presence of NFU1 (radical SAM assay), protein complex formation assay, Fe-S cluster transfer assay ACS bio & med chem Au High 36281303
2023 NFU1 functions in inserting the [4Fe-4S] cluster into the mitoribosome assembly factor METTL17, via the ISCA1-NFU1 node, thereby indirectly supporting mitoribosome small subunit assembly and mitochondrial protein synthesis. Fibroblasts from NFU1-deficient patients show previously unrecognized attenuation of mitochondrial protein synthesis. siRNA silencing of Fe-S cluster biosynthetic and delivery factors, mitoribosome assembly assays, mitochondrial translation assays, structure-function correlation studies, patient fibroblast analysis Nucleic acids research Medium 37823603
2023 Structural plasticity of NFU1's N-terminal domain is required for protein partner recognition during [4Fe-4S] cluster transfer. SAXS and paramagnetic NMR reveal structural models of ISCA1-ISCA2, ISCA1-ISCA2-NFU1, and ISCA1-NFU1 apo complexes; the terminal stable species is the ISCA1-NFU1 complex carrying the [4Fe-4S] cluster, and the N-terminal domain of NFU1 acts as a modulator of cluster transfer. Small-angle X-ray scattering (SEC-SAXS), paramagnetic NMR, structural modeling of apo and holo complexes Journal of molecular biology High 37211204
2017 The MMDS1 disease-causing Gly208Cys substitution in NFU1 increases propensity to dimerize and perturbs secondary structure, severely impairing the ability of NFU1 to accept a [4Fe-4S] cluster from physiologically relevant sources (ISCU, ISCA). The additional cysteine at position 208 does not contribute to cluster coordination; rather the structural change prevents cluster acquisition and downstream trafficking. In vitro biochemical characterization: protein stability assays, oligomeric state analysis (SEC), secondary structure analysis (CD spectroscopy), Fe-S cluster reconstitution assays, site-directed mutagenesis Journal of molecular biology High 28161430
2017 The MMDS1 disease-causing Gly189Arg substitution in NFU1 increases structural flexibility, decreases stability, and shifts the monomer-dimer equilibrium toward monomer, thereby impairing the ability of NFU1 to receive a [4Fe-4S] cluster from physiologically relevant sources. In vitro biochemical characterization: protein stability, oligomeric state, secondary structure (CD), Fe-S cluster reconstitution assays, site-directed mutagenesis The FEBS journal Medium 28906594
2003 The C-terminal NifU-like domain of the cytosolic protein HIRIP5 interacts with the N-terminal CBD-4 domain of laforin (EPM2A phosphatase) both in vitro (pull-down) and in vivo (co-immunoprecipitation); laforin dephosphorylates HIRIP5 in vitro, suggesting that NFU1/HIRIP5-related proteins participate in a phosphorylation-regulated interaction with laforin. Yeast two-hybrid, in vitro pulldown, co-immunoprecipitation, in vitro phosphatase assay Human molecular genetics Medium 12915448
2023 C. elegans nfu-1 (NFU1 ortholog) is required for normal acetylcholine signaling at neuromuscular junctions. Patient-specific point variants Gly147Arg and Gly166Cys cause allele-specific neuromuscular dysfunction: Gly147Arg causes hypersensitivity to acetylcholine at the presynaptic level (rescued by acetylcholine release knockdown), while Gly166Cys causes postsynaptic acetylcholine hypersensitivity. CRISPR knock-in of patient variants in C. elegans, acetylcholine sensitivity assays, genetic epistasis (knockdown of acetylcholine release), motility assays Disease models & mechanisms Medium 36645076

Source papers

Stage 0 corpus · 59 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2000 NifS-directed assembly of a transient [2Fe-2S] cluster within the NifU protein. Proceedings of the National Academy of Sciences of the United States of America 267 10639125
2011 A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins. American journal of human genetics 225 22077971
2011 Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes. American journal of human genetics 220 21944046
1994 nifU gene product from Azotobacter vinelandii is a homodimer that contains two identical [2Fe-2S] clusters. Biochemistry 129 7947754
2004 The Arabidopsis chloroplastic NifU-like protein CnfU, which can act as an iron-sulfur cluster scaffold protein, is required for biogenesis of ferredoxin and photosystem I. The Plant cell 117 15031412
2005 NifS-mediated assembly of [4Fe-4S] clusters in the N- and C-terminal domains of the NifU scaffold protein. Biochemistry 114 16185064
2004 Iron-sulfur cluster assembly: NifU-directed activation of the nitrogenase Fe protein. The Journal of biological chemistry 111 14993221
2016 Role of Nfu1 and Bol3 in iron-sulfur cluster transfer to mitochondrial clients. eLife 106 27532773
2000 Modular organization and identification of a mononuclear iron-binding site within the NifU protein. Journal of biological inorganic chemistry : JBIC : a publication of the Society of Biological Inorganic Chemistry 104 10819462
1989 Nitrogen fixation (nif) genes of the cyanobacterium Anabaena species strain PCC 7120. The nifB-fdxN-nifS-nifU operon. The Journal of biological chemistry 101 2553733
2000 Characterization of the NifU and NifS Fe-S cluster formation proteins essential for viability in Helicobacter pylori. Biochemistry 96 11123951
1992 The nifU, nifS and nifV gene products are required for activity of all three nitrogenases of Azotobacter vinelandii. Molecular & general genetics : MGG 88 1538703
2015 Clinical, biochemical, and genetic spectrum of seven patients with NFU1 deficiency. Frontiers in genetics 82 25918518
1996 A modular domain of NifU, a nitrogen fixation cluster protein, is highly conserved in evolution. Journal of molecular evolution 52 8875867
2007 Evidence for nifU and nifS participation in the biosynthesis of the iron-molybdenum cofactor of nitrogenase. The Journal of biological chemistry 46 17959596
2014 Cavitating leukoencephalopathy with multiple mitochondrial dysfunction syndrome and NFU1 mutations. Frontiers in genetics 44 25477904
2016 Structural/Functional Properties of Human NFU1, an Intermediate [4Fe-4S] Carrier in Human Mitochondrial Iron-Sulfur Cluster Biogenesis. Structure (London, England : 1993) 41 27818104
1992 Activator-independent formation of a closed complex between sigma 54-holoenzyme and nifH and nifU promoters of Klebsiella pneumoniae. Molecular microbiology 41 1495390
2014 Leukoencephalopathy with cysts and hyperglycinemia may result from NFU1 deficiency. Mitochondrion 38 24462778
2005 NifU and NifS are required for the maturation of nitrogenase and cannot replace the function of isc-gene products in Azotobacter vinelandii. Biochemical Society transactions 38 15667274
2003 The Lafora disease gene product laforin interacts with HIRIP5, a phylogenetically conserved protein containing a NifU-like domain. Human molecular genetics 37 12915448
1991 Organization and function of binding sites for the transcriptional activator NifA in the Klebsiella pneumoniae nifE and nifU promoters. Journal of molecular biology 37 1880804
2020 Rats with a Human Mutation of NFU1 Develop Pulmonary Hypertension. American journal of respiratory cell and molecular biology 33 31461310
1995 Characterization of nifB, nifS, and nifU genes in the cyanobacterium Anabaena variabilis: NifB is required for the vanadium-dependent nitrogenase. Journal of bacteriology 32 7883714
1990 Activation of the Klebsiella pneumoniae nifU promoter: identification of multiple and overlapping upstream NifA binding sites. Nucleic acids research 31 2186362
2015 New spastic paraplegia phenotype associated to mutation of NFU1. Orphanet journal of rare diseases 28 25758857
2023 BOLA3 and NFU1 link mitoribosome iron-sulfur cluster assembly to multiple mitochondrial dysfunctions syndrome. Nucleic acids research 26 37823603
1980 The preparation, efficacy and safety of 'antigenoid' vaccine NFU1 (S-L+) MRC toward prevention of herpes simplex virus infections in human subjects. Medical microbiology and immunology 25 6258035
2020 The plastidial Arabidopsis thaliana NFU1 protein binds and delivers [4Fe-4S] clusters to specific client proteins. The Journal of biological chemistry 22 31911438
2017 Understanding the Molecular Basis of Multiple Mitochondrial Dysfunctions Syndrome 1 (MMDS1)-Impact of a Disease-Causing Gly208Cys Substitution on Structure and Activity of NFU1 in the Fe/S Cluster Biosynthetic Pathway. Journal of molecular biology 22 28161430
2020 Assembly of the [4Fe-4S] cluster of NFU1 requires the coordinated donation of two [2Fe-2S] clusters from the scaffold proteins, ISCU2 and ISCA1. Human molecular genetics 20 32776106
2022 In Vitro Demonstration of Human Lipoyl Synthase Catalytic Activity in the Presence of NFU1. ACS bio & med chem Au 19 36281303
2020 Single Mutation in the NFU1 Gene Metabolically Reprograms Pulmonary Artery Smooth Muscle Cells. Arteriosclerosis, thrombosis, and vascular biology 18 33297749
2015 A leaky splicing mutation in NFU1 is associated with a particular biochemical phenotype. Consequences for the diagnosis. Mitochondrion 18 26688339
2020 ISCU interacts with NFU1, and ISCU[4Fe-4S] transfers its Fe-S cluster to NFU1 leading to the production of holo-NFU1. Journal of structural biology 16 32151725
2001 Identification of human and mouse HIRA-interacting protein-5 (HIRIP5), two mammalian representatives in a family of phylogenetically conserved proteins with a role in the biogenesis of Fe/S proteins. Biochimica et biophysica acta 16 11342215
1988 Further analysis of nitrogen fixation (nif) genes in Azotobacter chroococcum: identification and expression in Klebsiella pneumoniae of nifS, nifV, nifM, and nifB genes and localization of nifE/N-, nifU-, nifA- and fixABC-like genes. Journal of general microbiology 15 3053983
2017 Understanding the molecular basis for multiple mitochondrial dysfunctions syndrome 1 (MMDS1): impact of a disease-causing Gly189Arg substitution on NFU1. The FEBS journal 13 28906594
2020 A genetic mimic of cerebral palsy: Homozygous NFU1 mutation with marked intrafamilial phenotypic variation. Brain & development 12 32747156
2017 NFU1 -Related Disorders as Key Differential Diagnosis of Cavitating Leukoencephalopathy. Journal of pediatric genetics 12 29441221
2021 ISCA1 Orchestrates ISCA2 and NFU1 in the Maturation of Human Mitochondrial [4Fe-4S] Proteins. Journal of molecular biology 11 33711344
1988 Cloning of nifHD from Nostoc commune UTEX 584 and of a flanking region homologous to part of the Azotobacter vinelandii nifU gene. Journal of bacteriology 10 3133363
2017 Novel NFU1 Variants Induced MMDS Behaved as Special Leukodystrophy in Chinese Sufferers. Journal of molecular neuroscience : MN 9 28470589
2017 Analysis of NFU-1 metallocofactor binding-site substitutions-impacts on iron-sulfur cluster coordination and protein structure and function. The FEBS journal 8 28906593
2015 Involvement of thioredoxin on the scaffold activity of NifU in heterocyst cells of the diazotrophic cyanobacterium Anabaena sp. strain PCC 7120. Journal of biochemistry 8 25953913
2007 The NMR structure of the domain II of a chloroplastic NifU-like protein OsNifU1A. Journal of biomolecular NMR 8 17431550
1982 The polar effect on nifM of mutations in the nifU,-S,-V genes of Klebsiella pneumoniae depends on their plasmid or chromosomal location. Molecular & general genetics : MGG 8 6752663
2017 Nfu1 Mediated ROS Removal Caused by Cd Stress in Tegillarca granosa. Frontiers in physiology 5 29326599
2019 "Idiopathic" pulmonary arterial hypertension in early infancy: Excluding NFU1 deficiency. Annals of pediatric cardiology 4 31516295
2016 NFU1 gene mutation and mitochondrial disorders. Neurology India 4 27381105
2014 Mitochondrial Protein Nfu1 Influences Homeostasis of Essential Metals in the Human Fungal Pathogen Cryptococcus neoformans. Mycobiology 4 25606020
2023 Structural Plasticity of NFU1 Upon Interaction with Binding Partners: Insights into the Mitochondrial [4Fe-4S] Cluster Pathway. Journal of molecular biology 3 37211204
2017 [Analysis of NFU1 gene mutation in a Chinese family affected with multiple mitochondrial dysfunction syndrome]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 2 28186588
2026 The oxygen sensitivity of [4Fe-4S] clusters on the nitrogenase scaffold protein NifU. Journal of inorganic biochemistry 1 41529390
2023 Patient-specific variants of NFU1/NFU-1 disrupt cholinergic signaling in a model of multiple mitochondrial dysfunctions syndrome 1. Disease models & mechanisms 1 36645076
2025 Yeast models of mutations in NFU1 gene for biochemical characterization and drug screening. Biochemical and biophysical research communications 0 40233434
2024 Azotobacter vinelandii scaffold protein NifU transfers iron to NifQ as part of the iron-molybdenum cofactor biosynthesis pathway for nitrogenase. The Journal of biological chemistry 0 39442618
2008 Verification and tissue-specific expression of nifU-like gene from the amphioxus Branchiostoma belcheri. DNA sequence : the journal of DNA sequencing and mapping 0 18300158
2007 Verification and tissue-specific expression of nifU-like gene from the amphioxus Branchiostoma belcheri. DNA sequence : the journal of DNA sequencing and mapping 0 17654012