NFU1 Nfu1p [Saccharomyces cerevisiae S288C] - Gene

Summary

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. Involved in [4Fe-4S] cluster assembly. 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, Jul 2025]
Orthologs: all
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Chromosome XI - NC_001143.9 Genomic Context describing neighboring genes

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

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
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed

Process	Evidence Code	Pubs
involved_in [4Fe-4S] cluster assembly	IGI Inferred from Genetic Interaction more info	PubMed
involved_in [4Fe-4S] cluster assembly	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in [4Fe-4S] cluster assembly	IPI Inferred from Physical Interaction more info	PubMed
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	IBA Inferred from Biological aspect of Ancestor more info

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 more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info

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)

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