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Protein

Frataxin, mitochondrial

Gene

FH

Organism
Arabidopsis thaliana (Mouse-ear cress)
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 (PubMed:17092311). May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+ (PubMed:17092311). May be able to store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization (PubMed:17092311). Binds to the mitochondrial cysteine desulfurase NIFS1 and increases its activity (PubMed:22511606).2 Publications

Catalytic activityi

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

GO - Molecular functioni

GO - Biological processi

  • aerobic respiration Source: TAIR
  • cellular iron ion homeostasis Source: GO_Central
  • cellular response to oxidative stress Source: GO_Central
  • heme biosynthetic process Source: UniProtKB-KW
  • ion transport Source: UniProtKB-KW
  • iron incorporation into metallo-sulfur cluster Source: GO_Central
  • mitochondrion organization Source: GO_Central
  • regulation of iron-sulfur cluster assembly Source: TAIR
  • response to hydrogen peroxide Source: TAIR
  • response to oxidative stress Source: TAIR

Keywordsi

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

Enzyme and pathway databases

BioCyciARA:AT4G03240-MONOMER
ReactomeiR-ATH-1268020 Mitochondrial protein import
R-ATH-1362409 Mitochondrial iron-sulfur cluster biogenesis

Names & Taxonomyi

Protein namesi
Recommended name:
Frataxin, mitochondrial (EC:1.16.3.1)
Short name:
Fxn
Gene namesi
Name:FH
Ordered Locus Names:At4g03240
ORF Names:F4C21.17
OrganismiArabidopsis thaliana (Mouse-ear cress)
Taxonomic identifieri3702 [NCBI]
Taxonomic lineageiEukaryotaViridiplantaeStreptophytaEmbryophytaTracheophytaSpermatophytaMagnoliophytaeudicotyledonsGunneridaePentapetalaerosidsmalvidsBrassicalesBrassicaceaeCamelineaeArabidopsis
Proteomesi
  • UP000006548 Componenti: Chromosome 4

Organism-specific databases

AraportiAT4G03240
TAIRilocus:2125477 AT4G03240

Subcellular locationi

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

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_0000193926? – 187Frataxin, mitochondrial
Transit peptidei1 – ?MitochondrionSequence analysis

Proteomic databases

PaxDbiQ9ZR07

Expressioni

Gene expression databases

ExpressionAtlasiQ9ZR07 baseline and differential
GenevisibleiQ9ZR07 AT

Interactioni

Subunit structurei

Monomer. Oligomer (By similarity). Interacts with NIFS1 (PubMed:22511606).By similarity1 Publication

Protein-protein interaction databases

BioGridi13304, 1 interactor
STRINGi3702.AT4G03240.1

Structurei

3D structure databases

ProteinModelPortaliQ9ZR07
SMRiQ9ZR07
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the frataxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3413 Eukaryota
COG1965 LUCA
HOGENOMiHOG000190729
InParanoidiQ9ZR07
KOiK19054
OMAiDEYHRLA
OrthoDBiEOG09360PXI
PhylomeDBiQ9ZR07

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.

Q9ZR07-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MATASRFLLR KLPRFLKLSP TLLRSNGVRV SSNLIQDSIE PLDSFWRIGS
60 70 80 90 100
RIRHDSLTTR SFSSQGPASV DYSSVLQEEE FHKLANFTIN HLLEKIEDYG
110 120 130 140 150
DNVQIDGFDI DYGNEVLTLK LGSLGTYVLN KQTPNRQIWM SSPVSGPSRF
160 170 180
DWDRDANAWI YRRTEAKLHK LLEEELENLC GEPIQLS
Length:187
Mass (Da):21,430
Last modified:December 6, 2005 - v2
Checksum:iFAFA6A8AF7FD7534
GO

Sequence cautioni

The sequence AAD14452 differs from that shown. Reason: Erroneous gene model prediction.Curated
The sequence CAB77809 differs from that shown. Reason: Erroneous gene model prediction.Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY649366 mRNA Translation: AAU11485.1
AC005275 Genomic DNA Translation: AAD14452.1 Sequence problems.
AL161496 Genomic DNA Translation: CAB77809.1 Sequence problems.
CP002687 Genomic DNA Translation: AEE82296.1
BT014964 mRNA Translation: AAT47815.1
AK176448 mRNA Translation: BAD44211.1
AK176831 mRNA Translation: BAD44594.1
PIRiB85041
RefSeqiNP_192233.2, NM_116562.4
UniGeneiAt.50317

Genome annotation databases

EnsemblPlantsiAT4G03240.1; AT4G03240.1; AT4G03240
GeneIDi828013
GrameneiAT4G03240.1; AT4G03240.1; AT4G03240
KEGGiath:AT4G03240

Similar proteinsi

Entry informationi

Entry nameiFRDA_ARATH
AccessioniPrimary (citable) accession number: Q9ZR07
Secondary accession number(s): Q6GKV0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 1, 2000
Last sequence update: December 6, 2005
Last modified: April 25, 2018
This is version 113 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programPlant Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
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Main funding by: National Institutes of Health