YFH1 ferroxidase [Saccharomyces cerevisiae S288C] - Gene

Summary

Go to the top of the page Help

Official Symbol: YFH1
Official Full Name: ferroxidase
Primary source: SGD:S000002278
Locus tag: YDL120W
See related: AllianceGenome:SGD:S000002278; FungiDB:YDL120W; VEuPathDB:YDL120W
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Saccharomyces cerevisiae S288C (strain: S288C)
Lineage: Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Summary: Enables ferrous iron binding activity; ferroxidase activity; and iron chaperone activity. Involved in glutathione metabolic process; intracellular iron ion homeostasis; and iron-sulfur cluster assembly. Located in mitochondrial matrix. Used to study Friedreich ataxia; cancer; and iron metabolism disease. Human ortholog(s) of this gene implicated in Friedreich ataxia; Friedreich ataxia 1; glioblastoma; and type 2 diabetes mellitus. Orthologous to human FXN (frataxin). [provided by Alliance of Genome Resources, Dec 2024]
NEW: Try the new Gene table
Try the new Transcript table

Genomic context

Go to the top of the page Help

See YFH1 in Genome Data Viewer

Location:: chromosome: IV

Exon count:: 1

Sequence:: Chromosome: IV; NC_001136.10 (245923..246447)

Chromosome IV - NC_001136.10 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

Go to the top of the page Help

Go to reference sequence details

Genomic Sequence:: NC_001136.10

Go to nucleotide: Graphics FASTA GenBank

Bibliography

Go to the top of the page Help

GeneRIFs: Gene References Into Functions

What's a GeneRIF?

In the absence of Yfh1, Isu1 was found to exist in two forms, one mostly monomeric with limited tendency to dimerize, distinct structural features suggest that in physiological conditions the zinc-regulated abundance of monomeric vs. oligomeric Isu1 yields [Yfh1].[Isu1] complexes with different Isu1 configurations that afford unique functional properties for Fe-S cluster assembly and delivery.
Title: Zinc and the iron donor frataxin regulate oligomerization of the scaffold protein to form new Fe-S cluster assembly centers.
YFH1 is spontaneously unfolded at low and high temperatures.
Title: Essential dynamics of the cold denaturation: pressure and temperature effects in yeast frataxin.
Mutual exclusivity of cluster assembly factor (Nfs1/Yfh1) and cluster transfer factor (Jac1/Ssq1) binding to Isu has functional consequences for the transition from the assembly process to the transfer process during biogenesis of FeS cluster proteins.
Title: Overlapping binding sites of the frataxin homologue assembly factor and the heat shock protein 70 transfer factor on the Isu iron-sulfur cluster scaffold protein.
The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity.
Title: Frataxin directly stimulates mitochondrial cysteine desulfurase by exposing substrate-binding sites, and a mutant Fe-S cluster scaffold protein with frataxin-bypassing ability acts similarly.
At an iron-to-protein ratio of 2, the initially monomeric Yfh1 is converted to a trimeric form in solution.
Title: The molecular basis of iron-induced oligomerization of frataxin and the role of the ferroxidation reaction in oligomerization.
Frataxin (YFH1) deficiency in yeast cells leads to increased DNA base oxidation and requirement of Apn1 for DNA damage repair.
Title: Apn1 AP-endonuclease is essential for the repair of oxidatively damaged DNA bases in yeast frataxin-deficient cells.
The authors observe that the high- and low-temperature denatured states of Yfh1 have similar but not identical residual secondary structures, different kinetics and compactness and a remarkably different degree of hydration.
Title: The role of hydration in protein stability: comparison of the cold and heat unfolded states of Yfh1.
in yeast, frataxin-deficiency interferes with mitochondrial dynamics, which might therefore be relevant for the pathophysiology of Friedreich ataxia.
Title: Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells.
Secondary inactivation of Aat1 contributes to the amplification of the respiratory defect observed in Deltayfh1 cells.
Title: Inactivation of mitochondrial aspartate aminotransferase contributes to the respiratory deficit of yeast frataxin-deficient cells.
Metacaspase is associated with mitochondrial respiration, intracellular redox control and genomic stability.
Title: The yeast metacaspase is implicated in oxidative stress response in frataxin-deficient cells.

Submit: New GeneRIF Correction See all GeneRIFs (30)

Pathways from PubChem

Go to the top of the page

Interactions

Go to the top of the page Help

Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

Go to the top of the page Help

Homology

Orthologs from OrthoDB

Gene Ontology Provided by SGD

Function	Evidence Code	Pubs
enables 2 iron, 2 sulfur cluster binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferric iron binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferric iron binding	IEA Inferred from Electronic Annotation more info
enables ferrous iron binding	IBA Inferred from Biological aspect of Ancestor more info
enables ferrous iron binding	IDA Inferred from Direct Assay more info	PubMed
enables ferroxidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables ferroxidase activity	IDA Inferred from Direct Assay more info	PubMed
enables ferroxidase activity	IEA Inferred from Electronic Annotation more info
enables iron chaperone activity	IBA Inferred from Biological aspect of Ancestor more info
enables iron chaperone activity	IDA Inferred from Direct Assay more info	PubMed
enables iron chaperone activity	IGI Inferred from Genetic Interaction more info	PubMed
enables oxidoreductase activity	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in glutathione metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in heme biosynthetic process	IEA Inferred from Electronic Annotation more info
involved_in intracellular iron ion homeostasis	IDA Inferred from Direct Assay more info	PubMed
involved_in intracellular iron ion homeostasis	IEA Inferred from Electronic Annotation more info
involved_in intracellular iron ion homeostasis	IGI Inferred from Genetic Interaction more info	PubMed
involved_in intracellular iron ion homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in iron ion transport	IEA Inferred from Electronic Annotation more info
involved_in iron-sulfur cluster assembly	IBA Inferred from Biological aspect of Ancestor more info
involved_in iron-sulfur cluster assembly	IDA Inferred from Direct Assay more info	PubMed
involved_in iron-sulfur cluster assembly	IGI Inferred from Genetic Interaction more info	PubMed
involved_in iron-sulfur cluster assembly	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in iron-sulfur cluster assembly	IPI Inferred from Physical Interaction 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 more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	IEA Inferred from Electronic Annotation more info

General protein information

Go to the top of the page Help

Preferred Names: ferroxidase

NP_010163.1

EC 1.16.3.1

Mitochondrial matrix iron chaperone; oxidizes and stores iron; interacts with Isu1p to promote Fe-S cluster assembly; mutation results in multiple Fe/S-dependent enzyme deficiencies; human frataxin homolog FXN is mutated in Friedrich's ataxia; human FTL gene can complement yeast yfh1 null mutant