Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Frataxin homolog, mitochondrial

Gene

YFH1

Organism
Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Promotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. Plays a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+. Can store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization. May be involved in regulation of the mitochondrial electron transport chain.6 Publications

Catalytic activityi

4 Fe2+ + 4 H+ + O2 = 4 Fe3+ + 2 H2O.1 Publication

GO - Molecular functioni

  • ferric iron binding Source: InterPro
  • ferrous iron binding Source: SGD
  • ferroxidase activity Source: SGD
  • iron chaperone activity Source: SGD

GO - Biological processi

  • cellular iron ion homeostasis Source: SGD
  • glutathione metabolic process Source: SGD
  • heme biosynthetic process Source: UniProtKB-KW
  • ion transport Source: UniProtKB-KW
  • iron-sulfur cluster assembly Source: SGD
  • mitochondrial electron transport, succinate to ubiquinone Source: UniProtKB
  • protein targeting to mitochondrion Source: Reactome
  • response to iron(II) ion Source: UniProtKB
  • response to oxidative stress Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Heme biosynthesis, Ion transport, Iron storage, Iron transport, Transport

Keywords - Ligandi

Iron

Enzyme and pathway databases

BioCyciMetaCyc:G3O-29519-MONOMER.
YEAST:G3O-29519-MONOMER.
ReactomeiR-SCE-1268020. Mitochondrial protein import.
R-SCE-1362409. Mitochondrial iron-sulfur cluster biogenesis.

Names & Taxonomyi

Protein namesi
Recommended name:
Frataxin homolog, mitochondrial (EC:1.16.3.1)
Cleaved into the following chain:
Gene namesi
Name:YFH1
Ordered Locus Names:YDL120W
OrganismiSaccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast)
Taxonomic identifieri559292 [NCBI]
Taxonomic lineageiEukaryotaFungiDikaryaAscomycotaSaccharomycotinaSaccharomycetesSaccharomycetalesSaccharomycetaceaeSaccharomyces
Proteomesi
  • UP000002311 Componenti: Chromosome IV

Organism-specific databases

EuPathDBiFungiDB:YDL120W.
SGDiS000002278. YFH1.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: Reactome
  • mitochondrial intermembrane space Source: Reactome
  • mitochondrial matrix Source: SGD
  • mitochondrion Source: SGD
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi79D → A: Nearly abolishes ferroxidase avtivity, slows down oligomerization, impairs resistance to iron-catalyzed oxidative stress, no effect on Fe(2+) delivery and cell growth; when associated with A-82. 1 Publication1
Mutagenesisi82D → A: Nearly abolishes ferroxidase avtivity, slows down oligomerization, impairs resistance to iron-catalyzed oxidative stress, no effect on Fe(2+) delivery and cell growth; when associated with A-79. 1 Publication1
Mutagenesisi93E → A: Impairs oligomerization and iron mineralization. 1 Publication1
Mutagenesisi93E → A: Impairs resistance to iron-catalyzed oxidative stress, no effect on Fe(2+) delivery and cell growth; when associated with A-97 and A-103. 1 Publication1
Mutagenesisi97D → A: Impairs resistance to iron-catalyzed oxidative stress, no effect on Fe(2+) delivery and cell growth; when associated with A-93 and A-103. 1 Publication1
Mutagenesisi103E → A: Impairs resistance to iron-catalyzed oxidative stress, no effect on Fe(2+) delivery and cell growth; when associated with A-93 and A-97. 1 Publication1
Mutagenesisi122 – 124NKQ → ATA: Impairs cell growth, lowers activity of motochondrial iron-sulfur cluster-containing enzymes, no effect on iron binding and oligomerization. 1 Publication3
Mutagenesisi129Q → A: Impairs cell growth and lowers aconitase activity. 1 Publication1
Mutagenesisi130I → A: Impairs cell growth and lowers aconitase activity. 1 Publication1
Mutagenesisi131W → A: Impairs cell growth, lowers aconitase activity and strongly decreases interaction with ISU1. 1 Publication1
Mutagenesisi131W → F: Lowers aconitase activity and no effexct on interaction with ISU1. 1 Publication1
Mutagenesisi141R → A: Impairs cell growth and lowers aconitase activity. 1 Publication1

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 21MitochondrionCuratedAdd BLAST21
ChainiPRO_000001013522 – 174Frataxin homolog intermediate formAdd BLAST153
ChainiPRO_000001013652 – 174Frataxin homolog, mitochondrialAdd BLAST123

Post-translational modificationi

Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein.

Proteomic databases

MaxQBiQ07540.
PRIDEiQ07540.

Interactioni

Subunit structurei

Monomer; increments in mitochondrial iron uptake induce stepwise assembly of species ranging from trimers to 24-mers. Interacts with ISU1. Interacts with YHB1, SDH1, SDH2, AIM45 and CIR1.7 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
ISU1Q030204EBI-2206814,EBI-29901
YHB1P396762EBI-2206814,EBI-6905

Protein-protein interaction databases

BioGridi31943. 99 interactors.
DIPiDIP-7485N.
IntActiQ07540. 11 interactors.
MINTiMINT-4300743.

Structurei

Secondary structure

1174
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Turni64 – 67Combined sources4
Helixi76 – 88Combined sources13
Turni89 – 91Combined sources3
Beta strandi92 – 94Combined sources3
Beta strandi96 – 99Combined sources4
Beta strandi101 – 104Combined sources4
Beta strandi106 – 112Combined sources7
Turni114 – 116Combined sources3
Beta strandi119 – 122Combined sources4
Beta strandi126 – 128Combined sources3
Beta strandi131 – 134Combined sources4
Turni135 – 137Combined sources3
Beta strandi138 – 151Combined sources14
Turni152 – 154Combined sources3
Helixi158 – 171Combined sources14

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2FQLX-ray3.01A52-174[»]
2GA5NMR-A53-174[»]
3OEQX-ray2.96A52-174[»]
3OERX-ray3.20A52-174[»]
4EC2X-ray3.00A52-174[»]
5T0Velectron microscopy17.50A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P/Q/R/S/T/U/V/W/X52-172[»]
ProteinModelPortaliQ07540.
SMRiQ07540.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ07540.

Family & Domainsi

Sequence similaritiesi

Belongs to the frataxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
InParanoidiQ07540.
KOiK19054.
OMAiHIPEVEY.
OrthoDBiEOG092C5EQR.

Family and domain databases

Gene3Di3.30.920.10. 1 hit.
InterProiIPR017789. Frataxin.
IPR002908. Frataxin/CyaY.
IPR020895. Frataxin_CS.
[Graphical view]
PANTHERiPTHR16821. PTHR16821. 1 hit.
PfamiPF01491. Frataxin_Cyay. 1 hit.
[Graphical view]
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
SUPFAMiSSF55387. SSF55387. 1 hit.
TIGRFAMsiTIGR03421. FeS_CyaY. 1 hit.
TIGR03422. mito_frataxin. 1 hit.
PROSITEiPS01344. FRATAXIN_1. 1 hit.
PS50810. FRATAXIN_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q07540-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MIKRSLASLV RVSSVMGRRY MIAAAGGERA RFCPAVTNKK NHTVNTFQKR
60 70 80 90 100
FVESSTDGQV VPQEVLNLPL EKYHEEADDY LDHLLDSLEE LSEAHPDCIP
110 120 130 140 150
DVELSHGVMT LEIPAFGTYV INKQPPNKQI WLASPLSGPN RFDLLNGEWV
160 170
SLRNGTKLTD ILTEEVEKAI SKSQ
Length:174
Mass (Da):19,490
Last modified:November 1, 1996 - v1
Checksum:iAB1FF7478EF0E5D6
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z74168 Genomic DNA. Translation: CAA98688.1.
AY558160 Genomic DNA. Translation: AAS56486.1.
BK006938 Genomic DNA. Translation: DAA11740.1.
PIRiS67663.
RefSeqiNP_010163.1. NM_001180179.1.

Genome annotation databases

EnsemblFungiiYDL120W; YDL120W; YDL120W.
GeneIDi851437.
KEGGisce:YDL120W.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z74168 Genomic DNA. Translation: CAA98688.1.
AY558160 Genomic DNA. Translation: AAS56486.1.
BK006938 Genomic DNA. Translation: DAA11740.1.
PIRiS67663.
RefSeqiNP_010163.1. NM_001180179.1.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2FQLX-ray3.01A52-174[»]
2GA5NMR-A53-174[»]
3OEQX-ray2.96A52-174[»]
3OERX-ray3.20A52-174[»]
4EC2X-ray3.00A52-174[»]
5T0Velectron microscopy17.50A/B/C/D/E/F/G/H/I/J/K/L/M/N/O/P/Q/R/S/T/U/V/W/X52-172[»]
ProteinModelPortaliQ07540.
SMRiQ07540.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi31943. 99 interactors.
DIPiDIP-7485N.
IntActiQ07540. 11 interactors.
MINTiMINT-4300743.

Proteomic databases

MaxQBiQ07540.
PRIDEiQ07540.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsemblFungiiYDL120W; YDL120W; YDL120W.
GeneIDi851437.
KEGGisce:YDL120W.

Organism-specific databases

EuPathDBiFungiDB:YDL120W.
SGDiS000002278. YFH1.

Phylogenomic databases

GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
InParanoidiQ07540.
KOiK19054.
OMAiHIPEVEY.
OrthoDBiEOG092C5EQR.

Enzyme and pathway databases

BioCyciMetaCyc:G3O-29519-MONOMER.
YEAST:G3O-29519-MONOMER.
ReactomeiR-SCE-1268020. Mitochondrial protein import.
R-SCE-1362409. Mitochondrial iron-sulfur cluster biogenesis.

Miscellaneous databases

EvolutionaryTraceiQ07540.
PROiQ07540.

Family and domain databases

Gene3Di3.30.920.10. 1 hit.
InterProiIPR017789. Frataxin.
IPR002908. Frataxin/CyaY.
IPR020895. Frataxin_CS.
[Graphical view]
PANTHERiPTHR16821. PTHR16821. 1 hit.
PfamiPF01491. Frataxin_Cyay. 1 hit.
[Graphical view]
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
SUPFAMiSSF55387. SSF55387. 1 hit.
TIGRFAMsiTIGR03421. FeS_CyaY. 1 hit.
TIGR03422. mito_frataxin. 1 hit.
PROSITEiPS01344. FRATAXIN_1. 1 hit.
PS50810. FRATAXIN_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiFRDA_YEAST
AccessioniPrimary (citable) accession number: Q07540
Secondary accession number(s): D6VRN0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: November 1, 1996
Last modified: November 2, 2016
This is version 153 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programFungal Protein Annotation Program

Miscellaneousi

Miscellaneous

Present with 1560 molecules/cell in log phase SD medium.1 Publication

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  2. SIMILARITY comments
    Index of protein domains and families
  3. Yeast
    Yeast (Saccharomyces cerevisiae): entries, gene names and cross-references to SGD
  4. Yeast chromosome IV
    Yeast (Saccharomyces cerevisiae) chromosome IV: entries and gene names

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.