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    NFU1 Nfu1p [ Saccharomyces cerevisiae S288C ]

    Gene ID: 853826, updated on 18-Sep-2024

    Summary

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    Official Symbol
    NFU1
    Official Full Name
    Nfu1p
    Primary source
    SGD:S000001523
    Locus tag
    YKL040C
    See related
    AllianceGenome:SGD:S000001523; FungiDB:YKL040C; VEuPathDB:YKL040C
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Saccharomyces cerevisiae S288C (strain: S288C)
    Lineage
    Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
    Also known as
    NUB1
    Summary
    Predicted to enable 4 iron, 4 sulfur cluster binding activity and iron ion binding activity. Involved in intracellular sequestering of iron ion; iron-sulfur cluster assembly; and protein maturation by [4Fe-4S] cluster transfer. Located in mitochondrial matrix. Human ortholog(s) of this gene implicated in multiple mitochondrial dysfunctions syndrome 1. Orthologous to human NFU1 (NFU1 iron-sulfur cluster scaffold). [provided by Alliance of Genome Resources, Apr 2022]
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    Genomic context

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    See NFU1 in Genome Data Viewer
    Location:
    chromosome: XI
    Exon count:
    1
    Sequence:
    Chromosome: XI; NC_001143.9 (361058..361828, complement)

    Chromosome XI - NC_001143.9Genomic Context describing neighboring genes Neighboring gene Spc42p Neighboring gene ESCRT-III subunit protein VPS24 Neighboring gene Ptm1p Neighboring gene ncRNA

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_001143.9

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins. Navarro-Sastre A, et al. Am J Hum Genet, 2011 Nov 11. PMID 22077971, Free PMC Article
    2. Jac1, a mitochondrial J-type chaperone, is involved in the biogenesis of Fe/S clusters in Saccharomyces cerevisiae. Voisine C, et al. Proc Natl Acad Sci U S A, 2001 Feb 13. PMID 11171977, Free PMC Article
    3. Saccharomyces cerevisiae ISU1 and ISU2: members of a well-conserved gene family for iron-sulfur cluster assembly. Garland SA, et al. J Mol Biol, 1999 Dec 10. PMID 10588895
    4. Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae. Schilke B, et al. Proc Natl Acad Sci U S A, 1999 Aug 31. PMID 10468587, Free PMC Article
    5. Mitochondrial localization of Arabidopsis thaliana Isu Fe-S scaffold proteins. Léon S, et al. FEBS Lett, 2005 Mar 28. PMID 15792798

    See all (40) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Homology

    Gene Ontology Provided by SGD

    Function Evidence Code Pubs
    enables 4 iron, 4 sulfur cluster binding IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables iron ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables iron-sulfur cluster binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables molecular_function ND
    No biological Data available
    more info
    		  
    Process Evidence Code Pubs
    involved_in iron-sulfur cluster assembly IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in iron-sulfur cluster assembly IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in protein maturation by [4Fe-4S] cluster transfer IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in protein maturation by [4Fe-4S] cluster transfer IGI
    Inferred from Genetic Interaction
    more info
    		 PubMed 
    involved_in protein maturation by [4Fe-4S] cluster transfer IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in protein maturation by [4Fe-4S] cluster transfer IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    involved_in protein maturation by iron-sulfur cluster transfer IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    located_in mitochondrial matrix IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in mitochondrial matrix IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrion HDA PubMed 
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion IEA
    Inferred from Electronic Annotation
    more info
    		  

    General protein information

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    Preferred Names
    Nfu1p
    NP_012884.3
    • Protein involved in Fe-S cluster transfer to mitochondrial clients; protects [4Fe-4S] clusters from damage due to oxidative stress; acts along with Bol3 at a late step in the transfer of [4Fe-4S] clusters from the ISA complex to client proteins; Fe-S loaded homodimer at steady state; similar to NifU, a bacterial protein required for Fe/S cluster maturation; ortholog of the human NFU1, mutations of which are associated with Multiple Mitochondria Dysfunctions Syndrome (MMDS1)

    NCBI Reference Sequences (RefSeq)

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    Genome Annotation

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference assembly

    Genomic

    1. NC_001143.9 Reference assembly

      Range
      361058..361828 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001179606.3 → NP_012884.3  TPA: Nfu1p [Saccharomyces cerevisiae S288C]

      See identical proteins and their annotated locations for NP_012884.3

      Status: REVIEWED

      UniProtKB/Swiss-Prot
      D6VXP6, P32860
      UniProtKB/TrEMBL
      B3LR48, B5VMB6, C7GP33, C8ZCD0, G2WHZ1, N1P0N7
      Conserved Domains (2) summary
      smart00932
      Location:24 → 117
      Nfu_N; Scaffold protein Nfu/NifU N terminal
      COG0694
      Location:147 → 235
      NifU; Fe-S cluster biogenesis protein NfuA, 4Fe-4S-binding domain [Posttranslational modification, protein turnover, chaperones]
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    P32860.2

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