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Protein

Frataxin homolog, mitochondrial

Gene

YFH1

Organism
Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast)
Status
Reviewed-Annotation score: -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

Miscellaneous

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

Catalytic activityi

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

GO - Molecular functioni

  • 2 iron, 2 sulfur cluster binding Source: GO_Central
  • ferric iron binding Source: GO_Central
  • ferrous iron binding Source: SGD
  • ferroxidase activity Source: SGD
  • identical protein binding Source: IntAct
  • iron chaperone activity Source: SGD

GO - Biological processi

  • cellular iron ion homeostasis Source: SGD
  • cellular response to oxidative stress Source: GO_Central
  • glutathione metabolic process Source: SGD
  • heme biosynthetic process Source: UniProtKB-KW
  • ion transport Source: UniProtKB-KW
  • iron incorporation into metallo-sulfur cluster Source: GO_Central
  • iron-sulfur cluster assembly Source: SGD
  • mitochondrial electron transport, succinate to ubiquinone Source: UniProtKB
  • mitochondrion organization Source: GO_Central
  • response to iron(II) ion Source: UniProtKB
  • response to oxidative stress Source: UniProtKB

Keywordsi

Molecular functionOxidoreductase
Biological processHeme biosynthesis, Ion transport, Iron storage, Iron transport, Transport
LigandIron

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

Protein family/group databases

TCDBi9.B.21.1.2 the frataxin (frataxin) family

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

Extracellular region or secreted Cytosol Plasma membrane Cell wall Cytoskeleton Vacuole Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi79D → A: Nearly abolishes ferroxidase activity, 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 activity, 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 mitochondrial 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
PaxDbiQ07540
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

Show more details

GO - Molecular functioni

  • identical protein binding Source: IntAct

Protein-protein interaction databases

BioGridi31943, 104 interactors
DIPiDIP-7485N
IntActiQ07540, 11 interactors
MINTiQ07540
STRINGi4932.YDL120W

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[»]
5TREelectron microscopy15.60A/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
OMAiSGPNRFD
OrthoDBiEOG092C5EQR

Family and domain databases

Gene3Di3.30.920.10, 1 hit
InterProiView protein in InterPro
IPR017789 Frataxin
IPR002908 Frataxin/CyaY
IPR036524 Frataxin/CyaY_sf
IPR020895 Frataxin_CS
PANTHERiPTHR16821 PTHR16821, 1 hit
PfamiView protein in Pfam
PF01491 Frataxin_Cyay, 1 hit
SMARTiView protein in SMART
SM01219 Frataxin_Cyay, 1 hit
TIGRFAMsiTIGR03421 FeS_CyaY, 1 hit
TIGR03422 mito_frataxin, 1 hit
PROSITEiView protein in PROSITE
PS01344 FRATAXIN_1, 1 hit
PS50810 FRATAXIN_2, 1 hit

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

Similar proteinsi

Entry informationi

Entry nameiFRDA_YEAST
AccessioniPrimary (citable) accession number: Q07540
Secondary accession number(s): D6VRN0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: November 1, 1996
Last modified: May 23, 2018
This is version 166 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programFungal Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

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